Intraocular Tumors of Childhood

1 Intraocular Tumors of Childhood

? Four intraocular locations (ie, structures/tissues)

? 2 Intraocular Tumors of Childhood

Choroid Four intraocular locations (ie, structures/tissues)

RPE

Retina 3 Intraocular Tumors of Childhood

1) ?(JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells. 2) ? : Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma.Six tumors Locally invasiveof the iris/ciliarydeath. Tx: Enucleate. body 3) ?: Strong association with NF1. Lighter on dark irides; darker on light. 4) ?: Strong association with Down syndrome; 15% of non-Down pop. 5) ? : Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota. 6) ?: Can be pupillary, stromal, secondary.

Choroid

RPE

Retina 4 Intraocular Tumors of Childhood

Choroid

RPE

Retina 5 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a… nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by age 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 6 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by age 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 7 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by age 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 8 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by age 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 9 Intraocular Tumors of Childhood

JXG: Skin papules. The orangish color is classic 10 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by age 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 11 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by 2#

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 12 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 13 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 14 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 15 Intraocular Tumors of Childhood

JXG: Iris lesion 16 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 17 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 18 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 19 Intraocular Tumors of Childhood

JXG: Spontaneous hyphema 20 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 21 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 22 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 23 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

Choroid In what three ways are the iris nodules clinically significant? --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis Should JXG nodules be removed surgically? --If enough nodules are present, heterochromia iridis will result Only if the glaucoma is uncontrollable RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 24 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cysts: Can before be pupillary, age 1 stromal,year, and secondary. almost all by 2

When JXG iris nodules are present, are they uni-, or bilateral? Unilateral

Retina 25 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages are they uni-,’ or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 26 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Toutoneponym giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’two words are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 27 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 28 Intraocular Tumors of Childhood

Touton giant cells Foamy macrophages

JXG 29 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages are they uni-,’ or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 30 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages are they uni-,’ or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 31 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages are they uni-,’ or bilateral? Unilateral

ChoroidThis histology—’foamyIn what three macrophages’--is ways are the iris oftennodules described clinically with significant? other, equivalent --Theyterms. Whatare prone are they? to spontaneous bleeding, with subsequent Foamy = ‘lipidhyphema filled’ and secondary glaucoma Macrophages--They = ‘histiocytes’ are in the DDx as a ‘masquerade syndrome’ in peds uveitis --If enough nodules are present, heterochromia iridis will result The point being, the terms ‘foamy macrophages,’ ‘lipid-filled (or -laden) macrophages,’ RPE What is the natural history of JXG? ‘foamy histiocytes,’ etc,It is all self-limited, mean the usually same resolving thing, so by don’t age 5be years misled if you see one term when you’re expecting another Retina 32 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Choroid Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphema and secondary glaucoma with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If with enough bilateral nodules panuveitis? are present, heterochromiaFirst clue--more iridis forthcomingwill result And a hx of chronic migratory arthritis? Associated withRPE chronicWhat diarrhea? is the natural history of JXG? And CNS symptoms--seizures,It is self-limited, dementia, usually coma? resolving by age 5 years Whipple’s disease Retina 33 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Choroid Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphema and secondary glaucoma with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If with enough bilateral nodules panuveitis? are present, heterochromia iridis will result And a hx of chronic migratory arthritis? Clue #2 Associated withRPE chronicWhat diarrhea? is the natural history of JXG? And CNS symptoms--seizures,It is self-limited, dementia, usually coma? resolving by age 5 years Whipple’s disease Retina 34 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Choroid Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphema and secondary glaucoma with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If with enough bilateral nodules panuveitis? are present, heterochromia iridis will result And a hx of chronic migratory arthritis? RPE Associated with chronicWhat diarrhea? is the natural historyNeed of another?JXG? And CNS symptoms--seizures,It is self-limited, dementia, usually coma? resolving by age 5 years Whipple’s disease Retina 35 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Choroid Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphema and secondary glaucoma with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If with enough bilateral nodules panuveitis? are present, heterochromia iridis will result And a hx of chronic migratory arthritis? Associated withRPE chronicWhat diarrhea? is the natural history of JXG? And CNS symptoms--seizures,It is self-limited, dementia, usually coma? resolving by age Last5 years chance--answer is next! Whipple’s disease Retina 36 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Choroid Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphema and secondary glaucoma with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If with enough bilateral nodules panuveitis? are present, heterochromia iridis will result And a hx of chronic migratory arthritis? Associated withRPE chronicWhat diarrhea? is the natural history of JXG? And CNS symptoms--seizures,It is self-limited, dementia, usually coma? resolving by age 5 years Whipple’s disease Retina 37 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Choroid Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphema and secondary glaucoma with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If with enough bilateral nodules panuveitis? are present, heterochromia iridis will result And a hx of chronic migratory arthritis? RPE What is the natural history of JXG? Associated with chronic Broadlydiarrhea? speaking, what sort of condition is Whipple’s? It is self-limited, usually resolving by age 5 years And CNS symptoms--seizures,It is infectious dementia, coma? Whipple’s disease Retina What infection agent is responsible for Whipple’s? The bacterium Tropheryma whipplei 38 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Choroid Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphema and secondary glaucoma with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If with enough bilateral nodules panuveitis? are present, heterochromia iridis will result And a hx of chronic migratory arthritis? RPE What is the natural history of JXG? Associated with chronic Broadlydiarrhea? speaking, what sort of condition is Whipple’s? It is self-limited, usually resolving by age 5 years And CNS symptoms--seizures,It is infectious dementia, coma? Whipple’s disease Retina What infection agent is responsible for Whipple’s? The bacterium Tropheryma whipplei 39 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Choroid Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphema and secondary glaucoma with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If with enough bilateral nodules panuveitis? are present, heterochromia iridis will result And a hx of chronic migratory arthritis? RPE What is the natural history of JXG? Associated with chronic Broadlydiarrhea? speaking, what sort of condition is Whipple’s? It is self-limited, usually resolving by age 5 years And CNS symptoms--seizures,It is infectious dementia, coma? Whipple’s disease Retina What infection agent is responsible for Whipple’s? The bacterium Tropheryma whipplei 40 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Choroid Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphema and secondary glaucoma with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If with enough bilateral nodules panuveitis? are present, heterochromia iridis will result And a hx of chronic migratory arthritis? RPE What is the natural history of JXG? Associated with chronic Broadlydiarrhea? speaking, what sort of condition is Whipple’s? It is self-limited, usually resolving by age 5 years And CNS symptoms--seizures,It is infectious dementia, coma? Whipple’s disease Retina What infection agent is responsible for Whipple’s? The bacterium Tropheryma whipplei 41 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral

Choroid When foamy macrophages are found in a biopsy performed Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? on a Whipple’s pt, from what site was the biopsy collected? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphemaThe duodenum and secondary glaucoma with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If withWhat enough bilateral other nodules finding panuveitis? are will present,a duodenal heterochromia biopsy reveal? iridis will result And a hx of chronic migratoryThe presence arthritis? of acid-fast bacteria within macrophages RPE Whatlocated is the in naturalintestinal history villi of JXG? Associated with chronic Broadlydiarrhea? speaking, what sort of condition is Whipple’s? It is self-limited, usually resolving by age 5 years And CNS symptoms--seizures,It is infectious dementia, coma? Whipple’s disease Retina What infection agent is responsible for Whipple’s? The bacterium Tropheryma whipplei 42 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral

Choroid When foamy macrophages are found in a biopsy performed Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? on a Whipple’s pt, from what site was the biopsy collected? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphemaThe duodenum and secondary (remember, glaucoma they have GI issues) with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If withWhat enough bilateral other nodules finding panuveitis? are will present,a duodenal heterochromia biopsy reveal? iridis will result And a hx of chronic migratoryThe presence arthritis? of acid-fast bacteria within macrophages RPE Whatlocated is the in naturalintestinal history villi of JXG? Associated with chronic Broadlydiarrhea? speaking, what sort of condition is Whipple’s? It is self-limited, usually resolving by age 5 years And CNS symptoms--seizures,It is infectious dementia, coma? Whipple’s disease Retina What infection agent is responsible for Whipple’s? The bacterium Tropheryma whipplei Intraocular Tumors of Childhood

Whipple’s disease: Duodenal biopsy, high mag. The image shows the characteristic feature of foamy macrophages in the lamina propria. 44 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral

Choroid When foamy macrophages are found in a biopsy performed Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? on a Whipple’s pt, from what site was the biopsy collected? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphemaThe duodenum and secondary (remember, glaucoma they have GI issues) with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If withWhat enough bilateral other nodules finding panuveitis? are will present,a duodenal heterochromia biopsy reveal? iridis will result And a hx of chronic migratoryThe presence arthritis? of acid-fast bacteria within macrophages RPE Whatlocated is the in naturalintestinal history villi of JXG? Associated with chronic Broadlydiarrhea? speaking, what sort of condition is Whipple’s? It is self-limited, usually resolving by age 5 years And CNS symptoms--seizures,It is infectious dementia, coma? Whipple’s disease Retina What infection agent is responsible for Whipple’s? The bacterium Tropheryma whipplei 45 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral

Choroid When foamy macrophages are found in a biopsy performed Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? on a Whipple’s pt, from what site was the biopsy collected? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphemaThe duodenum and secondary (remember, glaucoma they have GI issues) with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If withWhat enough bilateral other nodules finding panuveitis? are will present,a duodenal heterochromia biopsy reveal? iridis will result And a hx of chronic migratoryThe presence arthritis? of PAS+stain bacteria within macrophages located RPE Whatin intestinal is the natural villi history of JXG? Associated with chronic Broadlydiarrhea? speaking, what sort of condition is Whipple’s? It is self-limited, usually resolving by age 5 years And CNS symptoms--seizures,It is infectious dementia, coma? Whipple’s disease Retina What infection agent is responsible for Whipple’s? The bacterium Tropheryma whipplei 46 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light. 4)As Brushfield orangish spots skin :papules Strong association with Down syndrome; 15% of non-Down pop. 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral

Choroid When foamy macrophages are found in a biopsy performed Speaking of ‘foamy macrophages’…In what three ways are the iris nodules clinically significant? on a Whipple’s pt, from what site was the biopsy collected? What dz comes to mind--They if, instead are prone of ato youngspontaneous child bleeding, with subsequent hyphemaThe duodenum and secondary (remember, glaucoma they have GI issues) with iris nodules, the pt--They in question are in the was DDx a as a ‘masquerade syndrome’ in peds uveitis middle-aged white guy--If withWhat enough bilateral other nodules finding panuveitis? are will present,a duodenal heterochromia biopsy reveal? iridis will result And a hx of chronic migratoryThe presence arthritis? of PAS+ bacteria within macrophages located RPE Whatin intestinal is the natural villi history of JXG? Associated with chronic Broadlydiarrhea? speaking, what sort of condition is Whipple’s? It is self-limited, usually resolving by age 5 years And CNS symptoms--seizures,It is infectious dementia, coma? Whipple’s disease Retina What infection agent is responsible for Whipple’s? The bacterium Tropheryma whipplei 47 Intraocular Tumors of Childhood

Small-intestine biopsy stained with periodic acid-Schiff. Note the numerous macrophages in the lamina propria (arrows). 48 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of Speaking of foamy macrophagesnonpigmented part epithelium deaux… of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. What dz comes to mind if,3) instead Lisch nodules of a :young Strong associationchild with NF1. Lighter on dark irides; darker on light. As orangish skin papules with iris nodules, the pt in 4)question Brushfield was spots a: Strong association with Down syndrome; 15% of non-Down pop. Adult with bilateral upper-lid5) Iris yellow mammillations lesions?: Tiny, numerous.Pic forthcoming Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? Xanthelsasma 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina Intraocular Tumors of Childhood

Condition? 50 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of Speaking of foamy macrophagesnonpigmented part epithelium deaux… of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. What dz comes to mind if,3) instead Lisch nodules of a :young Strong associationchild with NF1. Lighter on dark irides; darker on light. As orangish skin papules with iris nodules, the pt in 4)question Brushfield was spots a: Strong association with Down syndrome; 15% of non-Down pop. Adult with bilateral upper-lid5) Iris yellow mammillations lesions?: Tiny, numerous.Pic forthcoming Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? Xanthelsasma 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 51 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of Speaking of foamy macrophagesnonpigmented part epithelium deaux… of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. What dz comes to mind if,3) instead Lisch nodules of a :young Strong associationchild with NF1. Lighter on dark irides; darker on light. As orangish skin papules with iris nodules, the pt in 4)question Brushfield was spots a: Strong association with Down syndrome; 15% of non-Down pop. Adult with bilateral upper-lid5) Iris yellow mammillations lesions?: Tiny, numerous.Pic forthcoming Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? Xanthelsasma 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Are xanthelasmas a harbinger of elevated serum lipids? Choroid In what three ways are the iris nodules clinically significant? They can be, but in most cases the individual has normal lipid panels --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma Can they be congenital? --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis Yes, and when they are, they usually are a sign of lipid derangement --If enough nodules are present, heterochromia iridis will result

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 52 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of Speaking of foamy macrophagesnonpigmented part epithelium deaux… of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. What dz comes to mind if,3) instead Lisch nodules of a :young Strong associationchild with NF1. Lighter on dark irides; darker on light. As orangish skin papules with iris nodules, the pt in 4)question Brushfield was spots a: Strong association with Down syndrome; 15% of non-Down pop. Adult with bilateral upper-lid5) Iris yellow mammillations lesions?: Tiny, numerous.Pic forthcoming Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? Xanthelsasma 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Are xanthelasmas a harbinger of elevated serum lipids? Choroid In what three ways are the iris nodules clinically significant? They can be, but in most cases the individual has normal lipid panels --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma Can they be congenital? --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis Yes, and when they are, they usually are a sign of lipid derangement --If enough nodules are present, heterochromia iridis will result

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 53 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of Speaking of foamy macrophagesnonpigmented part epithelium deaux… of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. What dz comes to mind if,3) instead Lisch nodules of a :young Strong associationchild with NF1. Lighter on dark irides; darker on light. As orangish skin papules with iris nodules, the pt in 4)question Brushfield was spots a: Strong association with Down syndrome; 15% of non-Down pop. Adult with bilateral upper-lid5) Iris yellow mammillations lesions?: Tiny, numerous.Pic forthcoming Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? Xanthelsasma 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Are xanthelasmas a harbinger of elevated serum lipids? Choroid In what three ways are the iris nodules clinically significant? They can be, but in most cases the individual has normal lipid panels --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma Can they be congenital? --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis Yes, and when they are, they usually are a sign of lipid derangement --If enough nodules are present, heterochromia iridis will result

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 54 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regressesIn three words,by age 5. what Treat sort inflamma of conditiontion and IOP.is JXG? Path: Touton giant cells. 2)It Medulloepitheliomais a…nonneoplastic: Benign histiocytic but locally proliferation aggressive neoplasia of Speaking of foamy macrophagesnonpigmented part epithelium deaux… of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedHow doeshyphema JXG usuallyincreased present? IOPglaucoma. (Hint: It’s Locally not invasiveophthalmic)death. Tx: Enucleate. What dz comes to mind if,3) instead Lisch nodules of a :young Strong associationchild with NF1. Lighter on dark irides; darker on light. As orangish skin papules with iris nodules, the pt in 4)question Brushfield was spots a: Strong association with Down syndrome; 15% of non-Down pop. Adult with bilateral upper-lid5) Iris yellow mammillations lesions?: Tiny, numerous.Pic forthcoming Same color as iris. Weak association with NF1, NevusAt what of Ota. age does it present? Xanthelsasma 6)The Iris majority cystsWhat: Can arebefore be the pupillary, agetwo 1hallmar stromal,year, andks secondary. of almost JXG histology? all by 2 The presence of Touton giant cells When JXGThe irispresence nodules of are ’foamy present, macrophages’ are they uni-, or bilateral? Unilateral Are xanthelasmas a harbinger of elevated serum lipids? Choroid In what three ways are the iris nodules clinically significant? They can be, but in most cases the individual has normal lipid panels --They are prone to spontaneous bleeding, with subsequent hyphema and secondary glaucoma Can they be congenital? --They are in the DDx as a ‘masquerade syndrome’ in peds uveitis Yes, and when they are, they usually are a sign of lipid derangement --If enough nodules are present, heterochromia iridis will result

RPE What is the natural history of JXG? It is self-limited, usually resolving by age 5 years

Retina 55 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 56 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 57 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 58 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 59 Intraocular Tumors of Childhood

How does it present? What extremelyAs important an iris mass function along does with the one nonpigmented or more of the epi following: of the CB perform? It is responsible--Glaucoma for the creation of aqueous humor Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 60 Intraocular Tumors of Childhood

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 61 Intraocular Tumors of Childhood

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 62 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 63 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: -- Choroid -- --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 64 Intraocular Tumors of Childhood

Medulloepithelioma/diktyoma 65 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: -- Choroid -- --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 66 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 67 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 68 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it benign, or malignant? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 69 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it a benign, or malignant lesion? It is benign, but very aggressive locally

How is it managed? Retina Enucleation is usually required 70 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it a benign, or malignant lesion? It can have features of both. Either way, it is very locally aggressive.

How is it managed? Retina Enucleation is usually required 71 Intraocular Tumors of Childhood

Medulloepithelioma/diktyoma 72 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it a benign, or malignant lesion? It can have features of both. Either way, it is very locally aggressive.

How is it managed? RetinaIs a tendencyEnucleation to metastasize is usually one requiredof its ‘malignant features’? No, this lesion rarely metastasizes; it does its damage locally 73 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it a benign, or malignant lesion? It can have features of both. Either way, it is very locally aggressive.

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it a benign, or malignant lesion? It can have features of both. Either way, it is very locally aggressive. How is it managed? How aggressive is ‘very aggressive’? Retina Enucleation is usually required Aggressive enough to result in death 75 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it a benign, or malignant lesion? It can have features of both. Either way, it is very locally aggressive.

How is it managed? How aggressive is ‘very aggressive’? Retina Enucleation is usually required Aggressive enough to result in death 76 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it a benign, or malignant lesion? It can have features of both. Either way, it is very locally aggressive.

How is it managed? Retina Enucleation is usually required 77 Intraocular Tumors of Childhood

How does it present? As an iris mass along with one or more of the following: --Glaucoma Choroid --Hyphema --Sectoral cataract

Is it common, or rare? Very rare

RPE Is it a benign, or malignant lesion? It can have features of both. Either way, it is very locally aggressive.

How is it managed? Retina Enucleation is usually required 78 Intraocular Tumors of Childhood

Retina 79 Intraocular Tumors of Childhood

Retina 80 Intraocular Tumors of Childhood

Retina 81 Intraocular Tumors of Childhood

Retina 82 Intraocular Tumors of Childhood

Retina 83 Intraocular Tumors of Childhood

Retina 84 Intraocular Tumors of Childhood

Retina 85 Intraocular Tumors of Childhood

Retina 86 Intraocular Tumors of Childhood

Retina 87 Intraocular Tumors of Childhood

Retina 88 Intraocular Tumors of Childhood

Retina 89 Intraocular Tumors of Childhood

Retina 90 Intraocular Tumors of Childhood

Retina 91 Intraocular Tumors of Childhood

Retina 92 Intraocular Tumors of Childhood

Retina 93 Intraocular Tumors of Childhood

Retina 94 Intraocular Tumors of Childhood

Retina 95 Intraocular Tumors of Childhood

Retina 96 Intraocular Tumors of Childhood

Retina 97 Intraocular Tumors of Childhood

Retina 98 Intraocular Tumors of Childhood

Retina 99 Intraocular Tumors of Childhood

Retina 100 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of Most NF1 lesions are associatednonpigmented with epithelium one ofof CB. two Pre cellsents: types. Iris mass What before are age they? 10 years. Can Iris/Ciliary Body Melanocytesbleedhyphemaandincreasedneuroglial IOPglaucoma.cells Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots Strong association with Down syndrome; 15% of non-Down pop. Lisch nodules are 5)most Iris mammillationsstrongly associated: Tiny, numerous. with what Same congenital color as condition? iris. Weak association with NF1, NF1 Nevus of Ota Melanocytic lesions6) Iris cysts: Can be pupillary, stromal, secondaryNeuroglial lesions --Café Areau laitLisch spots nodules associatedGive with fourNF2? classic --Nodular neurofibromas --Axillary/inguinal freckles examples of each: --Plexiform neurofibromas Yes, but the relationship is far(YMMV weaker—Lisch of course) nodules occur in NF2, but so --Lischsporadically nodules that they are not expected --Optic glioma --Choroidal lesions Choroid --Prominent corneal nerves What is the prevalence of Lisch nodules in NF1? In what fundamentalThe rule-of-thumb way do these is that lesions Lisch di noduleffer (other prevalence than the equalscell type the of ageorigin, of theduh)? patient The melanocytictimeslesions 10. Thus, are 50%of no of clinical 5 year significance olds will have beyond them, establishing 60% of 6 year the olds, diagnosis, etc. At whereas the neuroglialage 10 andlesions beyond, are essentially associated 100% with of significant NF1 patients ocular have and/or Lisch systemic nodules. morbidity Are LischRPE nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Retina 101 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of Most NF1 lesions are associatednonpigmented with epithelium one ofof CB. two Pre cellsents: types. Iris mass What before are age they? 10 years. Can Iris/Ciliary Body Melanocytesbleedhyphemaandincreasedneuroglial IOPglaucoma.cells Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots Strong association with Down syndrome; 15% of non-Down pop. Lisch nodules are 5)most Iris mammillationsstrongly associated: Tiny, numerous. with what Same congenital color as condition? iris. Weak association with NF1, NF1 Nevus of Ota Melanocytic lesions6) Iris cysts: Can be pupillary, stromal, secondaryNeuroglial lesions --Café Areau laitLisch spots nodules associatedGive with fourNF2? classic --Nodular neurofibromas --Axillary/inguinal freckles examples of each: --Plexiform neurofibromas Yes, but the relationship is far(YMMV weaker—Lisch of course) nodules occur in NF2, but so --Lischsporadically nodules that they are not expected --Optic glioma --Choroidal lesions Choroid --Prominent corneal nerves What is the prevalence of Lisch nodules in NF1? In what fundamentalThe rule-of-thumb way do these is that lesions Lisch di noduleffer (other prevalence than the equalscell type the of ageorigin, of theduh)? patient The melanocyticm’cytic v N-Gtimeslesions 10. Thus, are 50%of no of clinical 5 year significance olds will have beyond them, establishing 60% of 6 year the olds, diagnosis, etc. At whereas the neuroglialagem’cytic 10 v N-G and lesionsbeyond, are essentially associated 100% with of significant NF1 patients ocular have and/or Lisch systemic nodules. morbidity Are LischRPE nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Retina 102 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of Most NF1 lesions are associatednonpigmented with epithelium one ofof CB. two Pre cellsents: types. Iris mass What before are age they? 10 years. Can Iris/Ciliary Body Melanocytesbleedhyphemaandincreasedneuroglial IOPglaucoma.cells Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots Strong association with Down syndrome; 15% of non-Down pop. Lisch nodules are 5)most Iris mammillationsstrongly associated: Tiny, numerous. with what Same congenital color as condition? iris. Weak association with NF1, NF1 Nevus of Ota Melanocytic lesions6) Iris cysts: Can be pupillary, stromal, secondaryNeuroglial lesions --Café Areau laitLisch spots nodules associatedGive with fourNF2? classic --Nodular neurofibromas --Axillary/inguinal freckles examples of each: --Plexiform neurofibromas Yes, but the relationship is far(YMMV weaker—Lisch of course) nodules occur in NF2, but so --Lischsporadically nodules that they are not expected --Optic glioma --Choroidal lesions Choroid --Prominent corneal nerves What is the prevalence of Lisch nodules in NF1? In what fundamentalThe rule-of-thumb way do these is that lesions Lisch di noduleffer (other prevalence than the equalscell type the of ageorigin, of theduh)? patient The melanocytictimeslesions 10. Thus, are 50%of no of clinical 5 year significance olds will have beyond them, establishing 60% of 6 year the olds, diagnosis, etc. At whereas the neuroglialage 10 and lesionsbeyond, are essentially associated 100% with of significant NF1 patients ocular have and/or Lisch systemic nodules. morbidity Are LischRPE nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Retina 103 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of Most NF1 lesions are associatednonpigmented with epithelium one ofof CB. two Pre cellsents: types. Iris mass What before are age they? 10 years. Can Iris/Ciliary Body Melanocytesbleedhyphema andincreasedneuroglial IOPglaucoma. cells Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots Strong association with Down syndrome; 15% of non-Down pop. Lisch nodules are 5)most Iris mammillationsstrongly associated: Tiny, numerous. with what Same congenital color as condition? iris. Weak association with NF1, NF1 Nevus of Ota Melanocytic lesions6) Iris cysts: Can be pupillary, stromal, secondaryNeuroglial lesions --Café Areau laitLisch spots nodules associatedGive with fourNF2? classic --Nodular neurofibromas --Axillary/inguinal freckles examples of each: --Plexiform neurofibromas Yes, but the relationship is far(YMMV weaker—Lisch of course) nodules occur in NF2, but so --Lischsporadically nodules that they are not expected --Optic glioma --Choroidal lesions Choroid --Prominent corneal nerves What is the prevalence of Lisch nodules in NF1? In what fundamentalThe rule-of-thumb way do these is that lesions Lisch di noduleffer (other prevalence than the equalscell type the of ageorigin, of theduh)? patient The melanocytictimes lesions 10. Thus,are 50% of no of clinical 5 year significanceolds will have beyond them, establishing60% of 6 year the olds, diagnos etc. Atis, whereas the neuroglialage 10 and lesionsbeyond, are essentially associated 100% with of significant NF1 patients ocular have and/or Lisch systemic nodules. morbidity Are LischRPE nodules clinically significant? No; their only significance is as a diagnostic marker for NF1

Retina (No question—proceed when ready) 104 Intraocular Tumors of Childhood

Retina 105 Intraocular Tumors of Childhood

Retina 106 Intraocular Tumors of Childhood

Retina 107 Intraocular Tumors of Childhood

Lisch nodules 108 Intraocular Tumors of Childhood

What is the prevalence of Brushfield spots in the Down population? At least 90%

ChoroidWhat is the clinical significance of Brushfield spots? They have none

When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? WolfflinRPE nodules

Retina 109 Intraocular Tumors of Childhood

What is the prevalence of Brushfield spots in the Down population? At least 90%

ChoroidWhat is the clinical significance of Brushfield spots? They have none

When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? WolfflinRPE nodules

Retina 110 Intraocular Tumors of Childhood

Brushfield spots 111 Intraocular Tumors of Childhood

What is the prevalence of Brushfield spots in the Down population? At least 90%

ChoroidWhat is the clinical significance of Brushfield spots? They have none

When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? WolfflinRPE nodules

Retina 112 Intraocular Tumors of Childhood

What is the prevalence of Brushfield spots in the Down population? At least 90%

ChoroidWhat is the clinical significance of Brushfield spots? They have none

When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? WolfflinRPE nodules

Retina 113 Intraocular Tumors of Childhood

What is the prevalence of Brushfield spots in the Down population? At least 90%

ChoroidWhat is the clinical significance of Brushfield spots? They have none

When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? WolfflinRPE nodules

Retina 114 Intraocular Tumors of Childhood

What is the prevalence of Brushfield spots in the Down population? At least 90%

ChoroidWhat is the clinical significance of Brushfield spots? They have none

When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? WolfflinRPE nodules

Retina 115 Intraocular Tumors of Childhood

What is the prevalence of Brushfield spots in the Down population? At least 90%

ChoroidWhat is the clinical significance of Brushfield spots? They have none

When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? WolfflinRPE nodules

Retina 116 Intraocular Tumors of Childhood

What is the prevalence of Brushfield spots in the Down population? At least 90%

ChoroidWhat is the clinical significance of Brushfield spots? They have none

When a clinically identical iris finding occurs in a non-Down individual, what are the lesions called? WolfflinRPE nodules

Retina 117 Intraocular Tumors of Childhood

Wolfflin nodules 118 Intraocular Tumors of Childhood

OK, then what are iris mammillations? ChoroidTiny pigmented nodules which, when present, are found in vast numbers diffusely scattered across the iris surface

Are they unilateral, or bilateral? Usually unilateral, but bilaterality occurs frequently enough that it can’t be usedRPE to rule them out Are they associated with NF1? Yes (albeit not nearly as strongly as Lisch nodules) Retina 119 Intraocular Tumors of Childhood

OK, then what are iris mammillations? ChoroidTiny pigmented nodules which, when present, are found in vast numbers diffusely scattered across the iris surface

OK, then what are iris mammillations? ChoroidTiny pigmented iris nodules which, when present, are found in vast numbers diffusely scattered across the iris surface

Iris mammilations 124 Intraocular Tumors of Childhood

OK, then what are iris mammillations? ChoroidTiny pigmented iris nodules which, when present, are found in vast numbers diffusely scattered across the iris surface

OK, then what are iris mammillations? ChoroidTiny pigmented iris nodules which, when present, are found in vast numbers diffusely scattered across the iris surface ‘Tiny pigmented iris nodules associated with NF1’--given this, how on earth are you supposed to differentiateAre they unilateral, between or Lisch bilateral? nodules and mammillations? By appearance.Usually Iris mammillations unilateral, but are bilaterality always the occurs same frequently color as tenoughhe rest ofth atthe it can’tiris. be In contrast, andused as statedto rule previously, them out Lisch nodules are lighter when the iris is dark, but darker whenRPE the iris is light. With what phakomatosis are they associated? NF1 (albeit not nearly as strongly as Lisch nodules) Retina 129 Intraocular Tumors of Childhood

OK, then what are iris mammillations? ChoroidTiny pigmented iris nodules which, when present, are found in vast numbers diffusely scattered across the iris surface ‘Tiny pigmented iris nodules associated with NF1’--given this, how on earth are you supposed to differentiateAre they unilateral, between or Lisch bilateral? nodules and mammillations? By appearance.Usually Iris mammillations unilateral, but are bilaterality always the occurs same frequently color as tenoughhe rest ofth atthe it can’tiris. be In contrast andused as stated to rule previously, them out Lisch nodules are lighter when the iris is dark, but darker whenRPE the iris is light. With what phakomatosis are they associated? NF1 (albeit not nearly as strongly as Lisch nodules) Retina 130 Intraocular Tumors of Childhood

OK, then what are iris mammillations? ChoroidTiny pigmented iris nodules which, when present, are found in vast numbers diffusely scattered across the iris surface

Are they unilateral, or bilateral? Usually unilateral, but bilaterality occurs frequently enough that it can’t be usedRPE to rule them out With what phakomatosis are they associated? NF1 (albeit not nearly as strongly as Lisch nodules) In addition to NF1, iris mammillations have another important association. What isRetina it? Oculodermal melanocytosis, aka nevus of Ota 131 Intraocular Tumors of Childhood

OK, then what are iris mammillations? ChoroidTiny pigmented iris nodules which, when present, are found in vast numbers diffusely scattered across the iris surface

Oculodermal melanocytosis (nevus of Ota) 134 Intraocular Tumors of Childhood

Choroid

RPE

Retina 135 Intraocular Tumors of Childhood

1) ? 2) ? Choroid 3) ? Five tumors of the choroid 4) ? 5) ?

RPE

Retina 136 Intraocular Tumors of Childhood

1) Nevus 2) Melanocytoma Choroid 3) Osteoma Five tumors of the choroid 4) Isolated/focal choroidal hemangioma 5) Diffuse choroidal hemangioma

RPE

Retina 137 Intraocular Tumors of Childhood

1) Nevus 2) Melanocytoma Choroid 3) Osteoma Five tumors of the choroid 4) Isolated/focal choroidal hemangioma 5) Diffuse choroidal hemangioma But not 6) ?

What common sort of choroidal tumor—common in adults—is absent from this list? Choroidal tumors arising as metastases from a nonocular primary. In adults, metastasis of solid tumors to theRPE uveal tract is common. It almost never happens in children.

If a child does suffer an ophthalmic metastasis, where does it tend to occur? The orbit Retina 138 Intraocular Tumors of Childhood

1) Nevus 2) Melanocytoma Choroid 3) Osteoma Five tumors of the choroid 4) Isolated/focal choroidal hemangioma 5) Diffuse choroidal hemangioma But not 6) Metastases

If a child does suffer an ophthalmic metastasis, where does it tend to occur? The orbit Retina 139 Intraocular Tumors of Childhood

1) Nevus 2) Melanocytoma Choroid 3) Osteoma Five tumors of the choroid 4) Isolated/focal choroidal hemangioma 5) Diffuse choroidal hemangioma But not 6) Metastases

If a child does suffer an ophthalmic metastasis, where does it tend to occur? The orbit Retina 140 Intraocular Tumors of Childhood

1) Nevus 2) Melanocytoma Choroid 3) Osteoma Five tumors of the choroid 4) Isolated/focal choroidal hemangioma 5) Diffuse choroidal hemangioma But not 6) Metastases

If a child does suffer an ophthalmic metastasis, where does it tend to occur? The orbit Retina 141 Intraocular Tumors of Childhood

1) Nevus: Common. Benign Note the factoids, then proceed 2) Melanocytoma Choroid 3) Osteoma 4) Isolated/focal choroidal hemangioma 5) Diffuse choroidal hemangioma

RPE

Retina 142 Intraocular Tumors of Childhood

1) Nevus: Common. BenignIs it unilateral, or bilateral? 2) Melanocytoma Choroid 3) Osteoma It is virtually always unilateral 4) Isolated/focal choroidal hemangioma 5) Diffuse choroidal hemangiomaIs there a racial predilection? No

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields .

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 143 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields .

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 144 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields .

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 145 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields .

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 146 Intraocular Tumors of Childhood

Melanocytoma 147 Intraocular Tumors of Childhood

1) Nevus: Common. BenignDoes it have a unilateral/bilateral predilection? 2) Melanocytoma Choroid 3) Osteoma It is virtually always unilateral 4) Isolated/focal choroidal hemangioma 5) Diffuse choroidal hemangiomaIs there a racial predilection? No

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields .

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 148 Intraocular Tumors of Childhood

1) Nevus: Common. BenignDoes it have a unilateral/bilateral predilection? 2) Melanocytoma Choroid 3) Osteoma Yes, it is virtually always unilateral 4) Isolated/focal choroidal hemangioma 5) Diffuse choroidal hemangiomaIs there a racial predilection? No

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields .

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 149 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields .

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 150 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields .

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 151 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields .

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 152 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields .

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 153 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visualtwo words fields .

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 154 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields.

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 155 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields.

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 156 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields.

Does melanocytoma have the potential to undergo Retina malignant transformation? Yes 157 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields.

Does melanocytoma have the potential to undergo Retina malignant transformation? ApproximatelyYes what percent of cases will transform? 1-2 158 Intraocular Tumors of Childhood

Does it affect visual acuity? RPE Only in a minority of cases. But in almost all cases, it does affect visual fields.

Does melanocytoma have the potential to undergo Retina malignant transformation? ApproximatelyYes what percent of cases will transform? 1-2 159 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

If significant vision loss occurs, what osteoma complication is usually the culprit? Choroidal neovascular1) membraneNevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare Choroid 3) Osteoma 4) Isolated/focal choroidal hemangioma 5) Diffuse choroidal hemangioma

RPE

Retina 160 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina Intraocular Tumors of Childhood

Osteoma 162 Intraocular Tumors of Childhood

buzzword describing a b-scan Osteoma: FP, and b-scan demonstrating ‘shadowing’finding illustrated above 163 Intraocular Tumors of Childhood

Osteoma: FP, and b-scan demonstrating ‘shadowing’ 164 Intraocular Tumors of Childhood

Osteoma: Another example Intraocular Tumors of Childhood

Osteomas (same pt, different cuts). Note how bright the lesions are 166 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 167 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 168 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 169 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 170 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 171 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in femalesM v F spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 172 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 173 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 174 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 175 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 176 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; Is it benign or malignant?regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Benign 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 177 Intraocular Tumors of Childhood

Osteoma with CNVM in a 13 y.o. female 178 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; True osteomasIs it benign are or indeedmalignant? regressesrare; however, by age secon5. Treatdary inflamma osteoma-liketion and IOP. lesions Path: Toutoncan be gi foundant cells in eyes withBenign what sorts of history?2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/CiliaryEyes that have Body suffered severebleed chronichyphema inflammationincreased IOP (especiallyglaucoma. if they Locally become invasive phthisical)death. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 179 In a word, what is a choroidal osteoma composed of? IntraocularBone Tumors of Childhood Is it common or rare? Very rare 1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; True osteomasIs it benign are or indeedmalignant? regressesrare; however, by age secon5. Treatdary inflamma osteoma-liketion and IOP. lesions Path: Toutoncan be gi foundant cells in eyes withBenign what sorts of history?2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/CiliaryEyes that have Body suffered severebleed chronichyphema inflammationincreased IOP (especiallyglaucoma. if they Locally become invasive phthisical)death. Tx: Enucleate Does there a gender 3)predilection? Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Yes, it is more common4) Brushfield in females spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Is it more typically foundNevus in ofpre-teens, Ota or teens? Teens 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set)

RPE

Retina 180 Intraocular Tumors of Childhood

What1) Nevus is its: Common. characteristic Benign pattern on a-scan ultrasonography? It2) isMelanocytoma one of ‘high :internalUsually juxtapapillary.reflectivity’ Malignant transformation extremely rare Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma 5) Diffuse choroidal hemangioma

RPE

Retina 181 Intraocular Tumors of Childhood

RPE

Retina 182 Intraocular Tumors of Childhood

RPE

Retina 183 Intraocular Tumors of Childhood

RPE

Retina 184 Intraocular Tumors of Childhood

RPE

Retina 185 Intraocular Tumors of Childhood

RPE

Retina 186 Intraocular Tumors of Childhood

RPE

Retina 187 Intraocular Tumors of Childhood

RPE

Retina 188 Intraocular Tumors of Childhood

Circumscribed choroidal hemangioma 189 Intraocular Tumors of Childhood

RPE

Retina 190 Intraocular Tumors of Childhood

RPE

Retina 191 Intraocular Tumors of Childhood

Circumscribed choroidal hemangioma: High internal reflectivity on a-scan 192 Intraocular Tumors of Childhood

By what other name is this lesion known? Circumscribed1) Juvenile xanthogranulomachoroidal hemangioma(JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells Is2) itMedulloepithelioma common, or rare?(aka diktyoma): Benign but locally aggressive neoplasia of Rarenonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate Is3) itLisch associated nodules :with Strong a systemic association condition, with NF1. Lie,ighter is it on syndromic? dark irides; darker on light No4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota How does it present? ‘High internal6) Iris reflectivity’--what cysts: Can be pupillary, other stromal, choroidal secondary lesion’s (see a-scan the Iris issues is kids slide-set) As a reddish-orange mass in the macula is described the same way? Choroidal nevus What1) Nevus is its: Common. characteristic Benign pattern on a-scan ultrasonography? It2) isMelanocytoma one of ‘high: internalUsually juxtapapillary. reflectivity Malignant’ transformation extremely rare Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma 5) Diffuse choroidal hemangioma

RPE

Retina 193 Intraocular Tumors of Childhood

RPE

Retina 194

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; About 50% regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryThe coloration Body is a verybleed red--muchhyphema moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 195

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

RPE

Retina 196

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 197

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 198

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 199

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 200

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota What is the hallmark skin finding6) Irisin SWS? cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) The port-wineCan thestain choroidal hemangioma be present bilaterally? Yes, but it’s uncommon In one word, what sort of lesion1) is Nevus the port-wine: Common. stain? Benign An angiomaDoes the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern When does it present? 5) Diffuse choroidal hemangioma At birth

What is the typical pattern of distribution? It comports to the distributionRPE of one or more divisions of CN5 Does it always present in this manner? No. Some cases will cross the midline of the face

All infants with SWSRetina have a port-wine stain. Do all infants with a port-wine stain have SWS? No 201

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota What is the hallmark skin finding6) Irisin SWS? cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) The port-wineCan thestain choroidal hemangioma be present bilaterally? Yes, but it’s uncommon In one word, what sort of lesion1) is Nevus the port-wine: Common. stain? Benign An angiomaDoes the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern When does it present? 5) Diffuse choroidal hemangioma At birth

All infants with SWSRetina have a port-wine stain. Do all infants with a port-wine stain have SWS? No 202 Intraocular Tumors of Childhood

Sturge-Weber: Port-wine stain 203

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota What is the hallmark skin finding6) Irisin SWS? cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) The port-wineCan thestain choroidal hemangioma be present bilaterally? Yes, but it’s uncommon In one word, what sort of lesion1) is Nevus the port-wine: Common. stain? Benign An angiomaDoes the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern When does it present? 5) Diffuse choroidal hemangioma At birth

All infants with SWSRetina have a port-wine stain. Do all infants with a port-wine stain have SWS? No 204

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light Encephalotrigeminal angiomatosis (you might also What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillationsangioma: Tiny,tosis numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota What is the hallmark skin finding6) Irisin SWS? cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) The port-wineCan thestain choroidal hemangioma be present bilaterally? Yes, but it’s uncommon In one word, what sort of lesion1) is Nevus the port-wine: Common. stain? Benign An angiomaDoes the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern When does it present? 5) Diffuse choroidal hemangioma At birth

All infants with SWSRetina have a port-wine stain. Do all infants with a port-wine stain have SWS? No 205

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota What is the hallmark skin finding6) Irisin SWS? cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) The port-wineCan thestain choroidal hemangioma be present bilaterally? Yes, but it’s uncommon In one word, what sort of lesion1) is Nevus the port-wine: Common. stain? Benign An angiomaDoes the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern When does it present? 5) Diffuse choroidal hemangioma At birth

All infants with SWSRetina have a port-wine stain. Do all infants with a port-wine stain have SWS? No 206

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota What is the hallmark skin finding6) Irisin SWS? cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) The port-wineCan thestain choroidal hemangioma be present bilaterally? Yes, but it’s uncommon In one word, what sort of lesion1) is Nevus the port-wine: Common. stain? Benign An angiomaDoes the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern When does it present? 5) Diffuse choroidal hemangioma At birth

All infants with SWSRetina have a port-wine stain. Do all infants with a port-wine stain have SWS? No 207 Intraocular Tumors of Childhood

Sturge-Weber: Port-wine stain 208

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota What is the hallmark skin finding6) Irisin SWS? cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) The port-wineCan thestain choroidal hemangioma be present bilaterally? Yes, but it’s uncommon In one word, what sort of lesion1) is Nevus the port-wine: Common. stain? Benign An angiomaDoes the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern When does it present? 5) Diffuse choroidal hemangioma At birth

All infants with SWSRetina have a port-wine stain. Do all infants with a port-wine stain have SWS? No 209

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota What is the hallmark skin finding6) Irisin SWS? cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) The port-wineCan thestain choroidal hemangioma be present bilaterally? Yes, but it’s uncommon In one word, what sort of lesion1) is Nevus the port-wine: Common. stain? Benign An angiomaDoes the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern When does it present? 5) Diffuse choroidal hemangioma At birth

All infants with SWSRetina have a port-wine stain. Do all infants with a port-wine stain have SWS? No 210

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota What is the hallmark skin finding6) Irisin SWS? cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) The port-wineCan thestain choroidal hemangioma be present bilaterally? Yes, but it’s uncommon In one word, what sort of lesion1) is Nevus the port-wine: Common. stain? Benign An angiomaDoes the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern When does it present? 5) Diffuse choroidal hemangioma At birth

All infants with SWSRetina have a port-wine stain. Do all infants with a port-wine stain have SWS? No 211

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota What is the hallmark skin finding6) Irisin SWS? cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) The port-wineCan thestain choroidal hemangioma be present bilaterally? Yes, but it’s uncommon In one word, what sort of lesion1) is Nevus the port-wine: Common. stain? Benign An angiomaDoes the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern When does it present? 5) Diffuse choroidal hemangioma At birth

What is the typical pattern of distribution? It comports to the distributionRPE of one or more divisions of CN5 Does it always present in this manner? No, some cases will cross the midline of the face

All infants with SWSRetina have a port-wine stain. Do all infants with a port-wine stain have SWS? No 212

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota What is the hallmark skin finding6) Irisin SWS? cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) The port-wineCan thestain choroidal hemangioma be present bilaterally? Yes, but it’s uncommon In one word, what sort of lesion1) is Nevus the port-wine: Common. stain? Benign An angiomaDoes the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern When does it present? 5) Diffuse choroidal hemangioma At birth

All infants with SWSRetina have a port-wine stain. Do all infants with a port-wine stain have SWS? No 213

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; In a word,About what 50% sort of conditionregresses is SWS? by age 5. Treat inflammation and IOP. Path: Touton giant cells A phakomatosis 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryWhat is Thethe noneponymouscoloration Body is a very namebleed red--much forhyphema SWS? moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate Encephalotrigeminal angiomatosis3) Lisch nodules (you might: Strong also association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? see encephalofacial or cerebrofacial5) Iris mammillations angiomatosis: Tiny, numerous.) Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota What is the hallmark skin finding6) Irisin SWS? cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) The port-wineCan thestain choroidal hemangioma be present bilaterally? Yes, but it’s uncommon In one word, what sort of lesion1) is Nevus the port-wine: Common. stain? Benign An angiomaDoes the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern When does it present? 5) Diffuse choroidal hemangioma At birth

All infants with SWSRetina have a port-wine stain. Do all infants with a port-wine stain have SWS? No 214

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

RPE

Retina 215

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; About half regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroid Iris massal hemangioma? before age 10 years. Can Iris/CiliaryThe coloration Body is a verybleed red--muchhyphema moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 216

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; About half regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroidal Iris mass hemangioma? before age 10 years. Can Iris/CiliaryThe coloration Body is a verybleed red--muchhyphema moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 217

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; About half regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroidal Iris mass hemangioma? before age 10 years. Can Iris/CiliaryThe coloration Body is a verybleed red,hyphema much moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 218 Intraocular Tumors of Childhood

Sturge-Weber: Tomato catsup fundus OD 219

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; About half regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroidal Iris mass hemangioma? before age 10 years. Can Iris/CiliaryThe coloration Body is a verybleed red,hyphema much moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 220

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; About half regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroidal Iris mass hemangioma? before age 10 years. Can Iris/CiliaryThe coloration Body is a verybleed red,hyphema much moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 221

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; About half regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroidal Iris mass hemangioma? before age 10 years. Can Iris/CiliaryThe coloration Body is a verybleed red,hyphema much moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 222

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; About half regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroidal Iris mass hemangioma? before age 10 years. Can Iris/CiliaryThe coloration Body is a verybleed red,hyphema much moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 223

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; About half regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroidal Iris mass hemangioma? before age 10 years. Can Iris/CiliaryThe coloration Body is a verybleed red,hyphema much moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 224

IntraocularWith what condition Tumors is the diffuse choroidalof Childhood hemangioma associated? Sturge-Weber syndrome (SWS)

Diffuse choroidal hemangioma1) Juvenile xanthogranulomais present in what(JXG): percent Nonneoplastic of SWS? histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; About half regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of What does the fundusnonpigmented look like in epithelium an eye with of CB. a diffuse Presents: choroidal Iris mass hemangioma? before age 10 years. Can Iris/CiliaryThe coloration Body is a verybleed red,hyphema much moreincreased so than IOP anglaucoma. unaffected Locally fundus invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light What food-related term4) Brushfield is used to spots describe: Strong the association fundus appearance with Down syndrome in SWS? 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, ‘Tomato catsup fundus’Nevus of Ota 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) Can the choroidal hemangioma be present bilaterally? Yes, but it’s uncommon 1) Nevus: Common. Benign Does the choroidal hemangio2) Melanocytomama have: Usually malignant juxtapapillary. potential? Malignant transformation extremely rare No Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma

RPE

Retina 225 Intraocular Tumors of Childhood

1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma: Unilateral. Found in Sturge-Weber syndrome

RPE 1) ?

Retina 226 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE)

Retina 227 Intraocular Tumors of Childhood

What is the clinical appearance of CHRPE? Flat, mainly1) black Nevus lesion(s): Common. ranging Benign in size from a 1 mm up to ~10 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare ChoroidIs it common,3) Osteoma or rare?: Benign bony tumor, most common in teen years, females. Risk of CNVM Common 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern 5) Diffuse choroidal hemangioma: Unilateral. Found in Sturge-Weber syndrome Is it a hamartoma or a choristoma? It is neither RPE 1) Congenital hypertrophy of the RPE (CHRPE)

Retina 228 Intraocular Tumors of Childhood

Retina 229 Intraocular Tumors of Childhood

CHRPE 230 Intraocular Tumors of Childhood

Retina 231 Intraocular Tumors of Childhood

Retina 232 Intraocular Tumors of Childhood

Retina 233 Intraocular Tumors of Childhood

Retina 234 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 235 Intraocular Tumors of Childhood

Solitary Grouped

CHRPE 238 Intraocular Tumors of Childhood

CHRPE: Bear tracks 241 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, TakeNevus careful of Ota note of the modifier ‘like’ here, because while 6) Iris cysts: Can be pupillary, stromal, secondary (see the Iris issues is kids slide-set) A CHRPE-like lesion is associatedCHRPE andwith athe potentially lesions fataassociatedl inherited with syndrome. Gardner What syndrome is the name (both eponymous and noneponymous)are ophthalmoscopically of this syndrome? similar, they are not the same! Familial adenomatous polyposis,1) Nevusaka Gardner: Common. syndrome Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristicsChoroid of a CHRPE-like3) Osteoma presentation: Benign bony inc tumor,rease most the likelihoodcommon in thatteen ityears, is a componentfemales. Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) --If the shape of the lesions is pisciform RPE 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 244 Intraocular Tumors of Childhood

CHRPE-like lesions of Gardner syndrome: Bilateral presentation 248 Intraocular Tumors of Childhood

CHRPE-like lesions of Gardner syndrome: Scattered distribution 251 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) --If the shape of the lesions is pisciform What does pisciform mean? ‘Fish-shaped’ RPE 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 254 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) --If the shape of the lesions is pisciform What does pisciform mean? It means ‘fish-shaped’ RPE 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 255 Intraocular Tumors of Childhood

CHRPE-like lesions of Gardner syndrome: Pisciform shape 256 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) --If the shape of the lesions is pisciform The tails of these fish-shaped lesions have two telltale (tell-tail?) 1) Congenital hypertrophy of the RPE (CHRPE) characteristics--whatRPE are they? -- CHRPE is characterized according to its presentation. In what two ways does it present? -- --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 257 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) --If the shape of the lesions is pisciform The tails of these fish-shaped lesions have two telltale (tell-tail?) 1) Congenital hypertrophy of the RPE (CHRPE) characteristics--whatRPE are they? --They areCHRPE hypopigmentedhypo- vsis hyperpigmented characterized according to its presentation. In what two ways does it present? --They point--Solitary towards CHRPE the opticlocation nerve in eye head --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 258 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) --If the shape of the lesions is pisciform The tails of these fish-shaped lesions have two telltale (tell-tail?) 1) Congenital hypertrophy of the RPE (CHRPE) characteristics--whatRPE are they? --They areCHRPE hypopigmented is characterized according to its presentation. In what two ways does it present? --They point--Solitary towards CHRPE the optic nerve head --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 259 Intraocular Tumors of Childhood

CHRPE-like lesions of Gardner syndrome: Hypopigmented tail pointing toward ONH 260 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristicsWhat is the of most a CHRPE-like clinically important presentation (and inc ominous)rease the component likelihood tothat Gardner it is a component syndrome? Pts Choroiddevelop thousands3) Osteoma of colonic: Benign polyps, bony a tumor,significant most commonnumber ino fteen which years, are females. malignant Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesionsOther are than scattered the colonic throughout and RPE multiple lesions, sectors what areof the the eyes findings (ie, notin Gardner ‘grouped’) syndrome? --If the shape--Benign of the tumorslesions of is the pisciform skin --Benign tumors of bone --Dental RPEanomalies 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 261 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristicsWhat is the of most a CHRPE-like clinically important presentation (and inc ominous)rease the component likelihood tothat Gardner it is a component syndrome? Pts Choroiddevelop thousands3) Osteoma of colonic: Benign polyps, bony a tumor,significant most commonnumber ino fteen which years, are females. malignant Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesionsOther are than scattered the colonic throughout and RPE multiple lesions, sectors what areof the the eyes findings (ie, notin Gardner ‘grouped’) syndrome? --If the shape--Benign of the tumorslesions of is the pisciform skin --Benign tumors of bone --Dental RPEanomalies 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 262 Intraocular Tumors of Childhood

Gardner syndrome: Colonic polyps 263 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristicsWhat is the of most a CHRPE-like clinically important presentation (and inc ominous)rease the component likelihood tothat Gardner it is a component syndrome? Pts Choroiddevelop thousands3) Osteoma of colonic: Benign polyps, bony atumor, significant most common number in teen of years,which females. are malignant Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome Other than colonic and RPEWhat findings, proportion what areof untreated the common Gardner stigmata syndrome of Gardner pts will syndrome? develop --If the lesions are scattered throughoutcolon multiple cancer? sectors of the eyes (ie, not ‘grouped’) --If the shape--Benign of the tumorslesions of is the pisciform skin --Benign tumors of bone All of them --Dental anomalies RPE 1) CongenitalBy what hypertrophy age will this of theoccur? RPE (CHRPE) Age 40, maybe a little later CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 264 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristicsWhat is the of most a CHRPE-like clinically important presentation (and inc ominous)rease the component likelihood tothat Gardner it is a component syndrome? Pts Choroiddevelop thousands3) Osteoma of colonic: Benign polyps, bony atumor, significant most common number in teen of years,which females. are malignant Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome Other than colonic and RPEWhat findings, proportion what areof untreated the common Gardner stigmata syndrome of Gardner pts will syndrome? develop --If the lesions are scattered throughoutcolon multiple cancer? sectors of the eyes (ie, not ‘grouped’) --If the shape--Benign of the tumorslesions of is the pisciform skin --Benign tumors of bone All of them --Dental anomalies RPE 1) CongenitalBy what hypertrophy age will this of theoccur? RPE (CHRPE) Age 40, maybe a little later CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 265 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristicsWhat is the of most a CHRPE-like clinically important presentation (and inc ominous)rease the component likelihood tothat Gardner it is a component syndrome? Pts Choroiddevelop thousands3) Osteoma of colonic: Benign polyps, bony atumor, significant most common number in teen of years,which females. are malignant Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome Other than colonic and RPEWhat findings, proportion what areof untreated the common Gardner stigmata syndrome of Gardner pts will syndrome? develop --If the lesions are scattered throughoutcolon multiple cancer? sectors of the eyes (ie, not ‘grouped’) --If the shape--Benign of the tumorslesions of is the pisciform skin --Benign tumors of bone All of them --Dental anomalies RPE 1) CongenitalBy what hypertrophy age will this of theoccur? RPE (CHRPE) Age 40, maybe a little later CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 266 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristicsWhat is the of most a CHRPE-like clinically important presentation (and inc ominous)rease the component likelihood tothat Gardner it is a component syndrome? Pts Choroiddevelop thousands3) Osteoma of colonic: Benign polyps, bony atumor, significant most common number in teen of years,which females. are malignant Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome Other than colonic and RPEWhat findings, proportion what areof untreated the common Gardner stigmata syndrome of Gardner pts will syndrome? develop --If the lesions are scattered throughoutcolon multiple cancer? sectors of the eyes (ie, not ‘grouped’) --If the shape--Benign of the tumorslesions of is the pisciform skin --Benign tumors of bone All of them --Dental anomalies RPE 1) CongenitalBy what hypertrophy age will this of theoccur? RPE (CHRPE) 40, maybe a little later CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 267 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristicsWhat is the of most a CHRPE-like clinically important presentation (and inc ominous)rease the component likelihood tothat Gardner it is a component syndrome? Pts Choroiddevelop thousands3) Osteoma of colonic: Benign polyps, bony atumor, significant most common number in teen of years,which females. are malignant Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almostWhat choroidal proportion always hemangioma unilate of untreatedral :) Unilateral.Gardner Found syndrome in Sturge-Weber pts will syndrome develop --If the lesionsOther are than scattered colonic throughoutand RPE findings, multiple what sectors are ofthe the common eyes (ie, stigmata not ‘grouped’) of Gardner syndrome? --Benign tumors of the skincolon cancer? --If the shape of the lesions is pisciformAll of them What is the treatment of choice? --Benign tumors of bone Prophylactic colectomy --Dental anomalies RPE 1) CongenitalBy what hypertrophy age will this of theoccur? RPE (CHRPE) 40, maybe a little later CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 268 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristicsWhat is the of most a CHRPE-like clinically important presentation (and inc ominous)rease the component likelihood tothat Gardner it is a component syndrome? Pts Choroiddevelop thousands3) Osteoma of colonic: Benign polyps, bony atumor, significant most common number in teen of years,which females. are malignant Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almostWhat choroidal proportion always hemangioma unilate of untreatedral :) Unilateral.Gardner Found syndrome in Sturge-Weber pts will syndrome develop --If the lesionsOther are than scattered colonic throughoutand RPE findings, multiple what sectors are ofthe the common eyes (ie, stigmata not ‘grouped’) of Gardner syndrome? --Benign tumors of the skincolon cancer? --If the shape of the lesions is pisciformAll of them What is the treatment of choice? --Benign tumors of bone Prophylactic colectomy --Dental anomalies RPE 1) CongenitalBy what hypertrophy age will this of theoccur? RPE (CHRPE) 40, maybe a little later CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 269 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristicsWhat is the of most a CHRPE-like clinically important presentation (and inc ominous)rease the component likelihood tothat Gardner it is a component syndrome? Pts Choroiddevelop thousands3) Osteoma of colonic: Benign polyps, bony a tumor,significant most commonnumber ino fteen which years, are females. malignant Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesionsOther are than scattered the colonic throughout and RPE multiple lesions, sectors what areof the the eyes findings (ie, notin Gardner ‘grouped’) syndrome? --If the shape-- of the lesions is pisciform -- -- RPE 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 270 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristicsWhat is the of most a CHRPE-like clinically important presentation (and inc ominous)rease the component likelihood tothat Gardner it is a component syndrome? Pts Choroiddevelop thousands3) Osteoma of colonic: Benign polyps, bony a tumor,significant most commonnumber ino fteen which years, are females. malignant Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesionsOther are than scattered the colonic throughout and RPE multiple lesions, sectors what areof the the eyes findings (ie, notin Gardner ‘grouped’) syndrome? --If the shape--Benign of the tumorslesions of is the pisciform skin --Benign tumors of bone --Dental RPEanomalies 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 271 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare What characteristicsWhat is the of most a CHRPE-like clinically important presentation (and inc ominous)rease the component likelihood tothat Gardner it is a component syndrome? Pts Choroiddevelop thousands3) Osteoma of colonic: Benign polyps, bony a tumor,significant most commonnumber ino fteen which years, are females. malignant Risk of CNVM of Gardner syndrome? 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesionsOther are than scattered the colonic throughout and RPE multiple lesions, sectors what areof the the eyes findings (ie, notin Gardner ‘grouped’) syndrome? --If the shape--Benign of the tumorslesions of is the pisciform skin --Benign tumors of bone --Dental RPEanomalies 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 272 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) MelanocytomaWhat is issue’ the main is: Usually m entioned,ophthalmic juxtapapillary. two mani syndromes Malignantfestation transformationofshould Muir-Torre come extremely syndrome?to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaMultipleWhat is :thesebaceous Benign other? bony lesions tumor, most of (but common not necessarily in teen years, limited females. to) Risk the o eyelidsf CNVM of Gardner syndrome?Choroid How is Muir-Torre pronounced? Muir-Torre syndrome and4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) DiffuseDoesmure is almost Muir-Torre (rhymeschoroidal always hemangiomawithpresent unilate ‘pure’) withral :) Unilateral.multipletore-AY adenomatous Found in Sturge-Weber polyps syndromeof the colon --If the lesions are scattered throughouta la Gardner multiple syndrome? sectors of the eyes (ie, not ‘grouped’) --If the shape of the lesions is pisciformNo; Muir-Torre is an example of a disease spectrum called Hereditary Non-Polyposis Colorectal Cancer RPE 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 277 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) MelanocytomaWhat is issue’ the main is: Usually m entioned,ophthalmic juxtapapillary. two mani syndromes Malignantfestation transformationofshould Muir-Torre come extremely syndrome?to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaMultipleWhat is :thesebaceous Benign other? bony lesions tumor, most of (but common not necessarily in teen years, limited females. to) Risk the o eyelidsf CNVM of Gardner syndrome?Choroid How is Muir-Torre pronounced? Muir-Torre syndrome and4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) DiffuseDoesmure is almost Muir-Torre (rhymeschoroidal always hemangiomawithpresent unilate ‘pure’) withral :) Unilateral.multipletore-AY adenomatous Found in Sturge-Weber polyps syndromeof the colon --If the lesions are scattered throughouta la Gardner multiple syndrome? sectors of the eyes (ie, not ‘grouped’) --If the shape of the lesions is pisciformNo; Muir-Torre is an example of a disease spectrum called Hereditary Non-Polyposis Colorectal Cancer RPE 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 278 Intraocular Tumors of Childhood

Multiple skin-colored to yellow–pink papules (arrows) on the face of a 64-year-old woman with a history of colon and cervical cancer. A skin biopsy confirmed a diagnosis of sebaceous adenoma resulting from Muir–Torre syndrome 279 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign What sorts of sebaceous lesions? When ‘colon cancer + ophthalmic2) MelanocytomaWhat is issue’ the main is: Usually m entioned,ophthalmic juxtapapillary. two mani syndromes Malignantfestation-- transformationofshould Muir-Torre come extremely syndrome?to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaMultipleWhat is :thesebaceous Benign other? bony tumor,lesions most of common(but-- not innecessarily teen years, females.limited to)Risk the of CNVMeyelids of Gardner syndrome?Choroid How is Muir-Torre pronounced? Muir-Torre syndrome and4) Isolated/focal choroidal hemangioma--: VeryBasal-cell rare. Characteristic carcinomas a -scanwith pattern --If it is bilateral (regular CHRPE5) DiffuseDoesmure is almost Muir-Torre (rhymeschoroidal always hemangiomawithpresent unilate ‘pure’) withral :) Unilateral.multipletore-AYsebaceous adenomatous Found in differentiation Sturge-Weber polyps syndromeof the colon a --If the lesions are scattered throughoutla Gardner multiple syndrome? sectors of the eyes (ie, not ‘grouped’) --If the shape of the lesions is pisciformNo; Muir-Torre is an example of a disease spectrum called Hereditary Non-Polyposis Colorectal Cancer RPE 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 280 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign What sorts of sebaceous lesions? When ‘colon cancer + ophthalmic2) MelanocytomaWhat is issue’ the main is: Usually m entioned,ophthalmic juxtapapillary. two mani syndromes Malignantfestation--Sebaceous-cell transformationofshould Muir-Torre come carcinomas extremely syndrome?to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaMultipleWhat is :thesebaceous Benign other? bony tumor,lesions most of common(but--Sebaceous-cell not innecessarily teen years, females.adenomaslimited to)Risk the of CNVMeyelids of Gardner syndrome?Choroid How is Muir-Torre pronounced? Muir-Torre syndrome and4) Isolated/focal choroidal hemangioma--Basal-cell: Very rare. Characteristic carcinomas a -scanwith pattern --If it is bilateral (regular CHRPE5) DiffuseDoesmure is almost Muir-Torre (rhymeschoroidal always hemangiomawithpresent unilate ‘pure’) withral :) Unilateral.multipletore-AYsebaceous adenomatous Found in differentiation Sturge-Weber polyps syndromeof the colon a --If the lesions are scattered throughoutla Gardner multiple syndrome? sectors of the eyes (ie, not ‘grouped’) --If the shape of the lesions is pisciformNo; Muir-Torre is an example of a disease spectrum called Hereditary Non-Polyposis Colorectal Cancer RPE 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 281 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) MelanocytomaWhat is issue’ the main is: Usually m entioned,ophthalmic juxtapapillary. two mani syndromes Malignantfestation transformationofshould Muir-Torre come extremely syndrome?to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaMultipleWhat is :thesebaceous Benign other? bony lesions tumor, most of (but common not necessarily in teen years, limited females. to) Risk the o eyelidsf CNVM of Gardner syndrome?Choroid How is Muir-Torre pronounced? Muir-Torre syndrome and4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) DiffuseDoesmure is almost Muir-Torre (rhymeschoroidal always hemangiomawithpresent unilate ‘pure’) withral :) Unilateral.multipletore-AY adenomatous Found in Sturge-Weber polyps syndromeof the colon --If the lesions are scattered throughouta la Gardner multiple syndrome? sectors of the eyes (ie, not ‘grouped’) --If the shape of the lesions is pisciformNo; Muir-Torre is an example of a disease spectrum called Hereditary Non-Polyposis Colorectal Cancer RPE 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 282 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) MelanocytomaWhat is issue’ the main is: Usually m entioned,ophthalmic juxtapapillary. two mani syndromes Malignantfestation transformationofshould Muir-Torre come extremely syndrome?to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaMultipleWhat is :thesebaceous Benign other? bony lesions tumor, most of (but common not necessarily in teen years, limited females. to) Risk the o eyelidsf CNVM of Gardner syndrome?Choroid How is Muir-Torre pronounced? Muir-Torre syndrome and4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) DiffuseDoesmure is almost Muir-Torre (rhymeschoroidal always hemangiomawithpresent unilate ‘pure’) withral :) Unilateral.multipletore-AY adenomatous Found in Sturge-Weber polyps syndromeof the colon --If the lesions are scattered throughouta la Gardner multiple syndrome? sectors of the eyes (ie, not ‘grouped’) --If the shape of the lesions is pisciformNo; Muir-Torre is an example of a disease spectrum called Hereditary Non-Polyposis Colorectal Cancer RPE 1) Congenital hypertrophy of the RPE (CHRPE) CHRPE is characterized according to its presentation. In what two ways does it present? --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 283 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) Melanocytoma issue’ is: Usually mentioned, juxtapapillary. three syndromes Malignant transformation should come extremely to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaWhat is :the Benign other? bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome?Choroid Muir-Torre syndrome and4) Peutz-Jeghers Isolated/focal choroidal syndrome hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesionsWhat are isscattered the main throughout ophthalmic multiple manifestation sectors of of Peutz-Jeghers the eyes (ie, not syndrome? ‘grouped’) --If the shapeSimple of the lesionslentigines is pisciformof (but not necessarily limited to) the eyelids

Does Peutz-JehgersRPE 1) Congenital present with hypertrophy multiple ofadenomatous the RPE (CHRPE) polyps of the colon a la Gardner syndrome? CHRPE is characterized according to its presentation. In what two ways does it present? Yes --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 286 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) Melanocytoma issue’ is: Usually mentioned, juxtapapillary. three syndromes Malignant transformation should come extremely to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaWhat is :the Benign other? bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome?Choroid Muir-Torre syndrome and4) Peutz-Jeghers Isolated/focal choroidal syndrome hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesionsWhat are isscattered the main throughout ophthalmic multiple manifestation sectors of of Peutz-Jeghers the eyes (ie, not syndrome? ‘grouped’) --If the shapeSimple of the lesionslentigines is pisciformof (but not necessarily limited to) the eyelids

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) Melanocytoma issue’ is: Usually mentioned, juxtapapillary. three syndromes Malignant transformation should come extremely to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaWhat is :the Benign other? bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome?Choroid Muir-Torre syndrome and4) Peutz-Jeghers Isolated/focal choroidal syndrome hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) What is the main ophthalmicWhat maniare simplefestation lentigines of Peutz-Jeghers? syndrome? --If the shape of the lesions is pisciform Simple lentigines of (butFlat not melanocytic necessarily lesions limited histologically to) the eyelids similar to ephelides

1) Congenital hypertrophy of the RPE (CHRPE) Does Peutz-JehgersRPE presentBy what with variant multiple of theadenomatous term ‘simple polyps lentigines’ of the are they colon a la Gardner syndrome? CHRPE is characterized accordingalso known? to its presentation. In what two ways does it present? Yes --Solitary CHRPE ‘Lentigo simplex’ --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina Does lentigo simplex have malignant potential? No 288 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) Melanocytoma issue’ is: Usually mentioned, juxtapapillary. three syndromes Malignant transformation should come extremely to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaWhat is :the Benign other? bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome?Choroid Muir-Torre syndrome and4) Peutz-Jeghers Isolated/focal choroidal syndrome hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) What is the main ophthalmicWhat maniare simplefestation lentigines of Peutz-Jeghers? syndrome? --If the shape of the lesions is pisciform Simple lentigines of (butFlat not melanocytic necessarily lesions limited histologically to) the eyelids similar to ephelides

1) Congenital hypertrophy of the RPE (CHRPE) Does Peutz-JehgersRPE presentBy what with variant multiple of theadenomatous term ‘simpleWhat polyps lentigines’ are ephelidesof the are they(singular, also ephelis)? colon a la Gardner syndrome? Freckles CHRPE is characterized accordingknown? to its presentation. In what two ways does it present? Yes --Solitary CHRPE ‘Lentigo simplex’ --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina Does lentigo simplex have malignant potential? No 290 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) Melanocytoma issue’ is: Usually mentioned, juxtapapillary. three syndromes Malignant transformation should come extremely to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaWhat is :the Benign other? bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome?Choroid Muir-Torre syndrome and4) Peutz-Jeghers Isolated/focal choroidal syndrome hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) What is the main ophthalmicWhat maniare simplefestation lentigines of Peutz-Jeghers? syndrome? --If the shape of the lesions is pisciform Simple lentigines of (butFlat not melanocytic necessarily lesions limited histologically to) the eyelids similar to ephelides

Peutz-Jeghers syndrome: Eyelid simple lentigines 292 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) Melanocytoma issue’ is: Usually mentioned, juxtapapillary. three syndromes Malignant transformation should come extremely to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaWhat is :the Benign other? bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome?Choroid Muir-Torre syndrome and4) Peutz-Jeghers Isolated/focal choroidal syndrome hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) What is the main ophthalmicWhat maniare simplefestation lentigines of Peutz-Jeghers? syndrome? --If the shape of the lesions is pisciform Simple lentigines of (butFlat not melanocytic necessarily lesions limited histologically to) the eyelids similar to ephelides

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) Melanocytoma issue’ is: Usually mentioned, juxtapapillary. three syndromes Malignant transformation should come extremely to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaWhat is :the Benign other? bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome?Choroid Muir-Torre syndrome and4) Peutz-Jeghers Isolated/focal choroidal syndrome hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) What is the main ophthalmicWhat maniare simplefestation lentigines of Peutz-Jeghers? syndrome? --If the shape of the lesions is pisciform Simple lentigines of (butFlat not melanocytic necessarily lesions limited histologically to) the eyelids similar to ephelides I coulda sworn lentigo simplex had malignant potential. 1) Congenital hypertrophy of the RPE (CHRPE) Does Peutz-JehgersRPE presentBy what withYou variant mu sureltiple ofabout theadenomatous termthis? ‘simple polyps lentigines’ of the are they colon a la Gardner syndrome?Yes, I’m sure. You’re thinking of lentigo maligna , CHRPE is characterized accordingalso known? to its presentation. In what two ways does it present? Yes a pre-malignant melanocytic lesion of the skin. --Solitary CHRPE ‘Lentigo simplex’ --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina Does lentigo simplex have malignant potential? No 297 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) Melanocytoma issue’ is: Usually mentioned, juxtapapillary. three syndromes Malignant transformation should come extremely to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaWhat is :the Benign other? bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome?Choroid Muir-Torre syndrome and4) Peutz-Jeghers Isolated/focal choroidal syndrome hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) What is the main ophthalmicWhat maniare simplefestation lentigines of Peutz-Jeghers? syndrome? --If the shape of the lesions is pisciform Simple lentigines of (butFlat not melanocytic necessarily lesions limited histologically to) the eyelids similar to ephelides I coulda sworn lentigo simplex had malignant potential. 1) Congenital hypertrophy of the RPE (CHRPE) Does Peutz-JehgersRPE presentBy what withYou variant mu sureltiple ofabout theadenomatous termthis? ‘simple polyps lentigines’ of the are they colon a la Gardner syndrome?Yes, I’m sure. You’re thinking of lentigotwo wordsmaligna , CHRPE is characterized accordingalso known? to its presentation. In what two ways does it present? Yes a pre-malignant melanocytic lesion of the skin. --Solitary CHRPE ‘Lentigo simplex’ --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina Does lentigo simplex have malignant potential? No 298 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) Melanocytoma issue’ is: Usually mentioned, juxtapapillary. three syndromes Malignant transformation should come extremely to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaWhat is :the Benign other? bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome?Choroid Muir-Torre syndrome and4) Peutz-Jeghers Isolated/focal choroidal syndrome hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesions are scattered throughout multiple sectors of the eyes (ie, not ‘grouped’) What is the main ophthalmicWhat maniare simplefestation lentigines of Peutz-Jeghers? syndrome? --If the shape of the lesions is pisciform Simple lentigines of (butFlat not melanocytic necessarily lesions limited histologically to) the eyelids similar to ephelides I coulda sworn lentigo simplex had malignant potential. 1) Congenital hypertrophy of the RPE (CHRPE) Does Peutz-JehgersRPE presentBy what withYou variant mu sureltiple ofabout theadenomatous termthis? ‘simple polyps lentigines’ of the are they colon a la Gardner syndrome?Yes, I’m sure. You’re thinking of lentigo maligna , CHRPE is characterized accordingalso known? to its presentation. In what two ways does it present? Yes a pre-malignant melanocytic lesion of the skin. --Solitary CHRPE ‘Lentigo simplex’ --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina Does lentigo simplex have malignant potential? No 299 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) Melanocytoma issue’ is: Usually mentioned, juxtapapillary. three syndromes Malignant transformation should come extremely to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaWhat is :the Benign other? bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome?Choroid Muir-Torre syndrome and4) Peutz-Jeghers Isolated/focal choroidal syndrome hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesionsWhat are isscattered the main throughout ophthalmic multiple manifestation sectors of of Peutz-Jeghers the eyes (ie, not syndrome? ‘grouped’) --If the shapeSimple of the lesions lentigines is pisciform of (but not necessarily limited to) the eyelids

Are lentigoDoes simplex Peutz-JehgersRPE eyelid1) lesions Congenital present the with hypertrophyclassic multiple harbinger ofadenomatous the ofRPE Peutz-Jehgers (CHRPE) polyps of syndrome?the No, pigmentedcolon a lalesions Gardner of the syndrome? perioral region are the classic/most common finding CHRPE is characterized according to its presentation. In what two ways does it present? Yes --Solitary CHRPE --Multifocal or Grouped CHRPE: Large lesion(s) surrounded by a few smaller ones Retina 300 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) Melanocytoma issue’ is: Usually mentioned, juxtapapillary. three syndromes Malignant transformation should come extremely to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaWhat is :the Benign other? bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome?Choroid Muir-Torre syndrome and4) Peutz-Jeghers Isolated/focal choroidal syndrome hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesionsWhat are isscattered the main throughout ophthalmic multiple manifestation sectors of of Peutz-Jeghers the eyes (ie, not syndrome? ‘grouped’) --If the shapeSimple of the lesions lentigines is pisciform of (but not necessarily limited to) the eyelids

Characteristic circumoral pigmentation in a patient with Peutz-Jeghers syndrome 303 Intraocular Tumors of Childhood

Speaking of: Did you notice the pigmented lip lesions in this pic? 304 Intraocular Tumors of Childhood

1) Juvenile xanthogranuloma (JXG): Nonneoplastic histiocytic proliferation. <2 years old. +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of nonpigmented epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreased IOPglaucoma. Locally invasivedeath. Tx: Enucleate 3) Lisch nodules: Strong association with NF1. Lighter on dark irides; darker on light 4) Brushfield spots: Strong association with Down syndrome 5) Iris mammillations: Tiny, numerous. Same color as iris. Weak association with NF1, Nevus of Ota A CHRPE-like lesion is associated6) Iris with cysts a :potentia Can be pupillary,lly fatal inheritedstromal, secondary syndrome. (see What the Iris is issues the name is kids slide-set) (both eponymous and noneponymous) of this syndrome? Familial adenomatous polyposis, aka Gardner syndrome 1) Nevus: Common. Benign When ‘colon cancer + ophthalmic2) Melanocytoma issue’ is: Usually mentioned, juxtapapillary. three syndromes Malignant transformation should come extremely to mind. rare What characteristics of a CHRPE-like presentation increase the likelihood that it is a component One is Gardner syndrome3). OsteomaWhat is :the Benign other? bony tumor, most common in teen years, females. Risk of CNVM of Gardner syndrome?Choroid Muir-Torre syndrome and4) Peutz-Jeghers Isolated/focal choroidal syndrome hemangioma: Very rare. Characteristic a-scan pattern --If it is bilateral (regular CHRPE5) Diffuse is almost choroidal always hemangioma unilateral :) Unilateral. Found in Sturge-Weber syndrome --If the lesionsWhat are isscattered the main throughout ophthalmic multiple manifestation sectors of of Peutz-Jeghers the eyes (ie, not syndrome? ‘grouped’) --If the shapeSimple of the lesionslentigines is pisciformof (but not necessarily limited to) the eyelids

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

Retina 1) ? 307 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 308 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) ?

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 309 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 310 Intraocular Tumors of Childhood

How does it present1) clinically?Nevus: Common. Benign As a variably pigmented,2) Melanocytoma slightly :elevatedUsually juxtapapillary. retinal mass Malignant of the transformation peripapillary extremely retina rare Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern With what more sinister5) Diffuse dz entitychoroidal is hemangiomait often confused?: Unilateral. Found in Sturge-Weber syndrome Choroidal melanoma--eyes have been enucleated because of this misdiagnosis

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 311 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 312 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 313 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 314 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) Intraocular Tumors of Childhood

What is the name of the reverse clinical entity, ie, one with normal cells found in 1)an Juvenile abnormal xanthogranulomalocation? (JXG): Nonneoplastic histiocytic proliferation. <2 years old. A choristoma +/- skin papules. Iris nodules bleedhyphemaincreased IOPglaucoma. Self-limited; regresses by age 5. Treat inflammation and IOP. Path: Touton giant cells 2) Medulloepithelioma (aka diktyoma): Benign but locally aggressive neoplasia of choristoma What is a hamartomanonpigmented? epithelium of CB. Presents: Iris mass before age 10 years. Can Iris/Ciliary Body bleedhyphemaincreasednormal IOPglaucoma. Locallyan invasive abnormaldeath. Tx: Enucleate A tumor composed 3)of Lisch histologically nodules: Strong abnormal association cells with NF1.found Lighter in th oneir dark normal irides; darker location on light 4) Brushfield spots: Strong association with Down syndrome So, what combination5) Iris of mammillations hamartomatous: Tiny, numerous. cells of the Same retina color asand iris. RPEWeak associationare involved with NF1in , That a lesion is Nevusa hamartoma of Ota (or choristoma) indicates what about its onset? a combinedThat it ishamartoma congenital6) Iris cystsof the: Can retina be pupillary, and RPE? stromal, secondary (see the Iris issues is kids slide-set) RPE cells (duh) and retinal glial cells That a lesion is a hamartoma (or choristoma) indicates what about its status How doesvis a visit present malignancy?1) clinically?Nevus: Common. Benign As a variablyThat it is pigmented,benign2) Melanocytoma slightly :elevatedUsually juxtapapillary. retinal mass Malignant of the transformation peripapillary extremely retina rare Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern With what more sinister5) Diffuse dz entitychoroidal is hemangiomait often confused?: Unilateral. Found in Sturge-Weber syndrome Choroidal melanoma--eyes have been enucleated because of this misdiagnosis

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 319 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 320 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 321 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 322 Intraocular Tumors of Childhood

How does it present1) clinically?Nevus: Common. Benign 2) Melanocytoma: Usually juxtapapillary. Malignant transformation extremely rare As a variably pigmented, slightly elevated retinal mass of the peripapillaryarea retina Choroid 3) Osteoma: Benign bony tumor, most common in teen years, females. Risk of CNVM 4) Isolated/focal choroidal hemangioma: Very rare. Characteristic a-scan pattern With what more sinister5) Diffuse dz entitychoroidal is hemangiomait often confused?: Unilateral. Found in Sturge-Weber syndrome Choroidal melanoma--eyes have been enucleated because of this misdiagnosis

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 323 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 324 Intraocular Tumors of Childhood

Combined hamartoma of retina and RPE 325 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 326 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 327 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 328 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE

Retina 1) Retinoblastoma (see the slide-set dedicated to it) 329 Intraocular Tumors of Childhood

Combined hamartoma of retina and RPE. Note the entire lesion is above Bruchs 330 Intraocular Tumors of Childhood

RPE 1) Congenital hypertrophy of the RPE (CHRPE): Common. Deeply pigmented. A CHRPE-like finding is associated with Gardner syndrome

1) Combined hamartoma of the retina and RPE: Benign, congenital retinal lesion

Retina 1) Retinoblastoma (see the slide-set dedicated to it)

No question—summary/review slide