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Home , Pterygium (conjunctiva)

– Definition

Bumps in the Night II  A New growth  From Greek Conjunctival  Benign or malignant  Tumor – synonym  A new and abnormal proliferation of cells serving no physiological function Associate Professor, Illinois College Of Optometry  Conjunctival or corneal Attending,  From anterior ocular tissues and coverings Illinois Institute © 20013

Risk Factors Rising Incidence

 Advanced age  Increased UV exposure  Male gender  Older population  Light complexion  Increases in outdoor activities  Repeated, intense sun exposure  Changes in clothing styles  Outdoor occupations  Changes in ozone protection  Cigarette smoking  Rising incidence of disease  H/o squamous cell carcinoma (head/)  HIV ~ 10-fold ↑ risk of conjunctival neoplasm  AIDS

Concerning Characteristics of Concerning Characteristics of Ocular Neoplasms Ocular Neoplasms

1. New or rapidly growing lesion 4. Changes in surface characteristics  Overall size  Crusting, ulceration, bleeding, oozing  Depth of tissue invasion 5. Adjacent tissue changes 2. Changes in size or shape  Swelling, redness, satellite lesions  Particularly irregular borders 6. Changes in or significant symptoms 3. Change in appearance  Tenderness pain or itching  Particularly color  Particularly increasing pigmentation

1 General Management Principles General Management Principles

1. History 3. Photo document  Document lesion history well 1. Critical for any level of suspicion 2. Observation 2. Or even if thought relatively safe?  Main management of choice for most benign,  Slit lamp camera asymptomatic tumors  Cheap hand-held digital camera 1. Describe 4. Follow closely 2. Draw  Frequent follow-ups for concerning lesions 3. Document well 5. Biopsy or refer  Measure lesion at largest diameters  Growth or change (color, vasculature, etc.)  When in doubt

Ocular Tumor Organization Conjunctival Neoplasms Benign Malignant Anatomical Location Tissue Involvement  Choristomas  Conjunctival  and adnexa  Epithelial  Conjunctival Cysts Intraepithelial  and  Melanocytic  Papilloma Neoplasia  Glandular  Phlyctenule  Carcinoma in situ  Squamous Cell  : ,  Vascular  Pterygium Carcinoma   Lymphoid  Capillary Hemangioma  Malignant Melanoma  Lacrimal based  Pyogenic Granuloma  Kaposi's Sarcoma  Metastatic  Lymphoma  Lymphoma  Metastatic Tumors

Red Eye in Sjögren's Syndrome in Sjögren's Syndrome

 68-year-old woman with 15 yrs Sjögren's  MRI – thickened left lateral rectus beyond  CC: itching attributed/treated as KCS orbital rim  6 Mo - painful eye with increasing redness  Conjunctival B Cell lymphoma (MALT)  Radiation with remission > 2 years

von Moos, R. et al. J Clin Oncol; 24:1011-1013 2006 von Moos, R. et al. J Clin Oncol; 24:1011-1013 2006

2 Ocular Tumor Organization Conjunctival Choristomas

Benign Neoplasms Anatomical Location  Choristoma contains tissue not normally  Choristomas  Epithelial  Conjunctival Cysts present at the site  Melanocytic  Papilloma  Phlyctenule  Congenital (or shortly after birth)  Glandular  Pterygium  Not hereditary  Vascular Malignant Neoplasms  Conjunctival Intraepithelial  Lymphoid Neoplasm  Lacrimal based  Carcinoma in situ  Squamous Cell  Metastatic Carcinoma

Caruncle Oncocytoma is a glandular tumor

Dermoid Osseous Choristoma

 Congenital, skin containing lesions  Very solid nodule encapsulating bone  Solid, isolated, white-yellow, focal mass  Usually super temporal  Usually at inferotemporally limbus  Calcium confirmed by ultrasound or CT  Often displays thin white hairs

Conjunctival Cysts Complex Choristoma (Epithelial Inclusion Cyst)

 Contain tissue: dermal appendages,  Formed spontaneously lacrimal gland tissue, cartilage, bone, etc.  Epithelial cells pushed intoforming epithelium lined sacs  Appearance based on majority of tissue  Stimuli: trauma, inflammation, surgery  Can be quite large  Contain clear fluid and cellular debris  Transilluminate  Management:  Observe  Excise completely

3 Conjunctival Papilloma Cyst Video (Squamous Papilloma)

 Benign  Human papiloma virus (HPV)  Fibrovascular, pink  Pedunculated or sessile

Papilloma Papilloma Treatment

Childhood/Young Older Adults 1. Monitor Adults  Limbus 2. Topical steroid  Bulbar and Fornix  Single, unilateral 3. Cimetidine (Tagamet)  Multiple, bilateral  Wide growth  H-2 receptor blocker  Often stable w/o  May be very large growth  Significant corneal  Small and conjunctival inv.  Benign  Weeping blood  Can mimic SCC

Papilloma in a Child, Case 1 Papilloma in a Child, Case 2

 11-year-old boy  Treated with Cimetidine  CC sudden conjunctival  Tagamet bleeding  H-2 receptor blocker  What is it?  2 mo: dramatic regression  8-year h/o diffuse conjunctival papillomas  4 mo: resolution  Failed excisional biopsy, cryotherapy and mitomycin C  Diffuse recurrence over entire conjunctiva

Am J Ophthalmol 1999;128:362–364 Am J Ophthalmol 1999;128:362–364

4 Phlyctenule Phlyctenule Video Phlyctenular

 Small, nodular, circumscribed limbal lesion  Lymphoid tissue  Ulcerates and heals 10 - 14 days  Delayed hypersensitivity to antigens  Mainly bacterial by-products  Associated with tuberculosis  Indicates altered immune function

Epithelial Based Carcinoma: Conjunctival Intraepithelial A Continuum Neoplasia (CIN)

1. Conjunctival Intraepithelial Conjunctival  Most common conjunctival malignancy Neoplasm (CIN) Intraepithelial rd Neoplasm  3 most common ocular tumor in geriatrics  Superficial thickened or atypical epithelium (pre-malignant)  Precancerous lesion 2. Carcinoma in situ Carcinoma in situ  Epithelial cells variably atypical, dysplastic  Full-thickness epithelium showing (Intraepithelial  At limbus malignant like cells neoplasia)  Can involve cornea  Intact basement membrane  Stain with Rose Bengal 3. SCC  Breaks through basement membrane Squamous cell  DDx , pterygium or pannus carcinoma  Invades conjunctival stroma

Common Presentations for Conjunctival Intraepithelial CIN, Carcinoma in Situ, SCC Neoplasia (CIN)

1. A raised vascular, gelatinous mass  Grey or slightly red 2. White, plaque-like lesion 3. Papilloma-like lesion 4. Opaque, membranous-like tissue spread onto peripheral cornea

5 Classification: Squamous Cell Squamous Cell Carcinoma Carcinoma of the Conjunctiva  Incidence of 1:800 1. Leukoplakic  Older, all races, both genders  White perilimbal plaque  Dilated peri-lesional blood vessels 2. Papillomatous  Signs of malignancy  Pinkish, limbal,  Bleeding corkscrew vesslels  Invasion of underlying tissues 3. Gelatinous  Dystal adhesion form limbus  Semi-translucent

Management: Squamous Cell Carcinoma Squamous Cell Carcinoma  In office management 1. Serial photography 2. Anterior segment ultrasound  R/o tissue invasion  Excision or biopsy criteria 1. If any of the characteristics described 2. Any focal conjunctival thickening with prominent abnormal vasculature

Squamous Cell Carcinoma Squamous Cell Carcinoma

6 Some Malignancies are Easier Squamous Cell Carcinoma to Diagnose Than Others

A Black Eye A Black Eye

 A77-year-old woman  Some progression, some regression  C/O OS had “turned black” past 6-9 mo  OS extensive pig, inc. lid margin and cornea  Primary acquired melanosis (PAM)of the conjunctiva with atypia (Confined to epithelium)  Followed q 2-6 mo.

Racial Melanosis Racial Melanosis (Melanocytosis) (Conjunctival Melanosis)

 Darkly pigmented persons  Flat bulbar conjunctival pigmentation  Usually  Bilateral  More dense at limbus  May extent on to cornea Axenfeld loop  Rarely progresses to melanoma

7 Racial Melanosis Congenital Melanocytosis

 Can spill over onto lid 1. Ocular  Eye only; lest prevalent 2. Dermal  Skin only; 1/3 of cases 3. Oculodermal (Nevus of Ota)

Nevus of Ota Nevus of Ota Oculodermal Congenital Melanocytosis  Skin & eye; most prevalent  Rarely this profound  Blue/grey pigment along 1st & 2nd  Note skin lesion along 1st & 2nd trigeminal trigeminal branches branches  Dark pigmented people, asians  Rare in whites

Congenital Ocular Melanocytosis Congenital Melanocytosis

 Congenital pigmentation of Associations scleral and/or peri-ocular tissues  Hyper perpigmentated  Easily confused w/ primary trabecular meshwork acquired melanosis (PAM)  Conjunctiva pigment  Heterochromia iridis  1:400 uveal melanoma risk  Iris mammillations  Not conjunctival melanoma  Usually whites  Dark fundus  Monitor every 6-12 months

8 Conjunctival Nevus Conjunctival Nevus

 Most common conjunctival  Unilateral melanocytic tumor  Visible by 2nd decade  Minimal to slight elevation  Don’t appear after 35  Varying pigment  Benign  Brown, reddish, amelanotic  < 1% conversion to malignant melanoma  Clear cysts common  Management  Photographic monitoring  Excision if documentable enlargement

Conjunctival Nevus Conjunctival Nevus

 Usually found 1. Limbal and perilimbal 2. Plica and caruncle 2nd most common location 3. Interpalpebral

Variously Pigmented Nevi Primary Acquired Melanosis (PAM)

 Middle age (vs nevus)  Common, 30% incidence in whites  Flat (vs nevus)  Diffuse and patchy  Moves easily with conjunctiva  Vs. racial/ocular melanosis

9 Primary Acquired Melanosis (PAM) Risks of Melanoma Associated with PAM

1. PAM without atypia General Classification Conjunctival  Rarely progresses to melanoma Melanoma Risk  ↑ pigmentation in epithelium w/ normal melanocytes PAM without atypia 0% 2. PAM with atypia PAM with atypia 46%  50%-90% conversion risk to malignant melanoma If atypical melanocytes show 75%  Atypical melanocytes abundant cytoplasm If atypical epithelial 90% melanocytes located in other than basal layer Folberg R. McLean IW, Zimmerman LE: Primary acquired melanosis of the conjunctiva. Hum Pathol 16:136. 1985. Adapted form Duanes

Primary Acquired Melanosis (PAM) Indications for Biopsy

Suspicious Signs Suspicious Locations  Focal, thickened, darker  Palpebral conjunctiva brown areas  Fornix  Darkly pigmented  Caruncle  Rapidly progressive  Distal to limbus  Large (bulbar conjunctiva)  Multifocal

Malignant Melanoma Suspicious PAM of the Conjunctiva  Typically Whites in the 5th decade  Very Rare  2% of ocular malignancies  4 new cases per 10 million per year in whites  8 X < for blacks  3 fold ↑ incidence 1973 - 1999 (white males)  Origins  10% from nevi  20% de novo  70% from PAM with atypia

10 Malignant Melanoma Conjunctival Melanoma Characteristics

 Strong metastatic tendencies  Localized  Primarily lymph nodes  Pre-auricular and submanddibular node palpation  Limbal & interpalpebral  Grow superficially for extended times  Pigmented  Rarely invade uvea or  But can be amelanotic  Prognosis Melanoma w/ abnormal vessels   25% overall mortality Prominent abnormal  Up to 45% if the tumor arose from PAM blood vessels  Many fold mortality rate if  Nodular 1 to > 10 mm  >0.8 mm thickness  lymphatic invasion

Melanoma developing from PAM

Conjunctival Melanoma Amelanotic Conjunctival Melanoma

Which is the melanoma? Capillary Hemangioma

 Benign  Congenital or develop near birth  May enlarge over several months  Often spontaneously resolves  Management  Observation  Surgical resection  Local or systemic Both! prednisone

11 Pyogenic Granuloma Pyogenic Granuloma

 Misnomer  Fast growing  Not pyogenic – puss forming (bacterial)  Pedunculated (stalk)  Inflammation not granulomatous  Red  Chronic inflammatory process from  Fleshy  Chalazia, foreign body,  Surgery: chalazia, pterygium, , etc.  Treatment  Steroid drops often successful  May require surgical excision

Pyogenic Granuloma Pyogenic Granuloma Video

 From FB  Topical steroids for a few weeks

Pyogenic Granuloma Video Conjunctival Neoplasms Formed over a  Epithelium Benign  Melanocytic  Capillary  Glandular Hemangioma  Vascular  Pyogenic Granuloma  Lymphoid Malignant  Lacrimal based  Kaposi’s sarcoma  Metastatic

12 Kaposi’s Sarcoma Kaposi's Sarcoma of the Conjunctiva  Most common HIV related ocular tumor  Quick growing neoplasms  Seen in healthy people w/ a rare sarcoma  Capillary related  70% of ocular lesions occur on the eyelids  Bright to dark red  Conjunctival lesions most common in  Spontaneous inferior fornix bleeding  Differentials include possible  Hemangioma, pyogenic granuloma, other

Kaposi's Sarcoma Lymphoid Tumors

 Very rare  Variable consequence  Benign - more common in children  Malignant - more common Mid to later age  Rarely sight or globe threatening  Most local, but can indicate systemic lymphoma  Most slow growing  AIDS associated fast growing

Lymphoid Tumors Lymphoid Tumors

 Minimal symptoms  Local ocular lesions are manifestations of systemic disease  Subepithelial mass  “Salmon patch” termed from salmon  Smooth color  Pink  Usually located in the fornix and stroma  At bulbar conjunctiva  Management or fornix 1. Must biopsy as cannot clinically differentiate  Minimal vascular benign versus malignant involvement 2. Must do systemic workup on all patients

13 Risk of Systemic Lymphoma Development based on Ocular Involvement Lymphoid tumors

% Systemic Lymphoma Development 1 Year 5 Year 10 Year

Lymphoid 7% 15% 28% Tumor Lymphoma 12% 38% 79% specifically

Shields CL, Shields JA. Carvalho C, et al: Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma. 108:979, 2001. Adapted from: Duane’s Ophthalmology Online, 2008 V4, Ch. 10

Management of Orbital Fat Prolapse Lymphoid Tumors  Complete history and review of systems  Biopsy  Blood workup  CT or MRI of torso to rule out systemic lymphoma  Treatment dependent on malignancy  Observation, steroids, radiation, chemotherapy

Metastatic Carcinoma Conjunctival Neoplasms of the Conjunctiva  Epithelium Malignant  Rare and predominantly breast cancer  Also skin melanoma and other 1o tumors  Melanocytic  Lung  Usually flashlight, pink and vascularized in the  Glandular  Most common in men conjunctiva stroma  Vascular  Breast  Metastatic melanoma is usually pigmented  Very rare  Lymphoid  Most common in  Lacrimal based women Conjunctival  Metastatic metastatic breast cancers

14 Concerning Characteristics of Management Principles Ocular Neoplasms 1. New or rapidly growing lesion 1. History  Size and depth  Document lesion history well 2. Changes in size or shape 2. Observation – describe, draw, document  Irregular borders  Main management for most benign, asymptomatic 3. Change in appearance, particularly color tumors  Increasing pigmentation 3. Photo document – critical if any suspicion  Even lesions thought to be safe? 4. Changes in the surface 4. Follow closely – Close interval if concerned  Crusting, ulceration, bleeding, oozing 5. Adjacent tissue changes 3. Biopsy or refer – When in doubt  Swelling, redness, satellite lesions  Growth or change 6. Changes in or significant symptoms  Just plain concerned

Thank You

David Castells, O.D. [email protected]

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