Update on Sellar & Orbital Imaging

Gabriella Szatmáry, MD, PhD Director of Neuro-Ophthalmology Neuroimager Hattiesburg Clinic, PA

American Society of Neuroimaging 36th Annual Meeting 01/19/2013 2 Intra +/-Suprasellar 1. Adenoma 2. Abscess 3. Craniopharyngioma 4. Eosinophilic granuloma 5. Hyperplasia 6. Langerhans cell histio 7. Lymphocytic hypophysitis 8. Mets 9. RCC (pars intermedia ) 10. Granular cell tumor or choristoma (post pit)

01/19/2013 3 Ectopic Neurohypophysis

A B

Pre- (A) and post-Gd (B) sagittal T1 images show “bright spot” in tuber cinereum of the hypothalamus. Post pit. hyperintesity may be lost in DI. 4 9/2008

4/2008 2/2008 Pituitary apoplexy 86 y/o VA↓, anorexia Sellar lesions: Adenoma (15% of all IC tumors) enlarge bony sella (macro>10mm)

77 y/o ♂ diplopia ↑PRL 28 x XRT Rx Dostinex, Steroids L-thyroxin

“Stalk effect” “Hook effect”

Pituitary abscess 28-year-old man with febrile cephalalgia

7 Lymphocytic Hypophysitis

Idiopathic inflammation of the pituitary gland Thickened, non tapering stalk (>2mm) Intense and homogeneous enhancement F:M = 8:1 8 Infundibular lesions

9 13 y/o ♀ VA ↓

SITA Standard 24-2 O.S.

O.D. Rathke’s Cleft Cyst (RCC)

RCC: intracystic nodule (mucin clump) with T1↑& T2↓ Derived from remnants of Rathke’s pouch.

Byun et al. AJNR Am J Neuroradiol 21:485–488, March 2000 11 LANGERHANS CELL HISTIOCYTOSIS X

24 y/o ♂ with DI Bx of scalp and groin lesion T12 & pit mets Proliferative do. of Langerhan’s cells Granular cell tumour

Sag T1WI C+ shows a well defined enhancing mass within the pituitary stalk. The pituitary gland is normal, as is the hypothalamus. Arise from stalk or neuro- hypophysis. Benign lesions, also called pituicytoma or choristoma. (Courtesy of L. Mechtler) 13 Pituitary protocol

Image Plane Sequence Slice / Gap FOV

Sagittal T1 3mm / 0mm 12 x 12

Coronal T1 3mm / 0mm 12 x 12

Coronal T2 3mm / 0mm 12 x 12

*Coronal Dynamic (pituitary TSE/FSE 3mm / 0mm 12 x 12 gland) T1+C

Sagittal T1+C 3mm / 0mm 12 x 12

Coronal T1+C Fat Sat 3mm / 0mm 12 x 12

Axial whole T1+C Fat Sat 12 x 12 brain 14 Extrasellar: Suprasellar

Craniopharyngioma Tuber cinereum hamartoma Arachnoid cyst Dermoid, Epidermoid Inflammation-infection Vascular

15 Craniopharyngioma epithelial: adamantinous or squamous-papillary

Sartoretti-Schefer et al. Am J Neuroradiol 18:77–87, 16 Germinoma  80-90% along IIIrd ma ventricle  5-10% basal ggl  Bifocal germinoma: pineal + suprasellar

 DWI restriction: high cellularity  Intense homogeneous Gad+  ± CSF seeding,  ± brain invasion 17

Hypothalamic/Optic Chiasm Gliomas

15-30% associated with NF-1

18 Tuber Cinereum Hamartoma

Post–Gd T1, T2 sagittal, and post-Gd T1 axial images. A mass is seen between the infundibulum and mammillary bodies, hyperintense to gray matter on T2. The mass appears similar to the brain parenchyma on T1 and shows no enhancement. This is a grey matter heterotopia. Large sessile causes gelastic seizures, and smaller pedunculated central precocious puberty. (courtesy of Dr. A. Osborn and Mechtler) 19 Arachnoid cyst in the suprasellar region markedly elevating the hypothalamus & stretching the pituitary stalk

elevation of the right side of the chiasm by the cyst no cyst wall is evident pituitary tissue itself is nl.  Same signal as CSF  No enhancement  No Ca++ 20 Colloid cysts are slow-growing lesions, close to foramen of Monro, rarely intra or suprasellar cistern, lined with epithelial cells and filled with mucus. Myriad of MRI signal: T1↑ T2→↓.

21 Dermoid Cyst

 <0.5% if primary intracranial tumors  Ectopic cyst containing dermal elements  Less common than epidermoid  Rupture causes significant morbidity and mortality

22 EPIDERMOID CYST

-1% of all IC tumors -At CPA, parasellar, 4th ventricular regions -Fast-FLAIR & DWI best sequences

23 45 Y/O WOMAN WITH CONFUSION

Neurosarcoidosis Solitary or multifocal CNS masses, dural plaque, parenchymal (non-caseating granulomas) 24  abnl. CXR in >90% of NS  WM small vessel vasculitis/angiitis  Coats CNs/fill IAC

 Leptomeningeal dz of the base of the brain similar to TB  spreads along the Virchow- Robin spaces to form intraparenchymal masses 25 Extrasellar: Parasellar Meningioma Schwannoma THS

CCF Cavernous hemang Thrombosis Lemierre syn

26 Patient presents with severe periorbital or retro-orbital pain of acute onset that is constant in nature; Diplopia due to opthalmoparesis usually follows the onset of the pain; The symptoms are usually unilateral 27 The International Headache Society criteria for THS  One or more episodes of unilateral orbital pain lasting for an average of 8 weeks if left untreated  Third, forth, and/or sixth cranial nerve palsy, which begins within two weeks of the onset of the orbital pain  Symptoms that resolve within 48-72 hours of initiation of steroid therapy  Exclusion of other etiologies by appropriate investigation, including neuroimaging.

28 Dx? B

29 29 40 y/o ♀ galactorrhea, PRL: 150

30 Multiple Myeloma 73 y/o ♂ IgA MM 2 yrs s/p stem cell transplant;

Confusion Headaches

Intrasellar recurrent MM extension to left cavernous sinus

PET/CT ↑F18 FDG uptake

31 Extrasellar: Infrasellar

32 Chordoma

Chordomas are the most common lesions of the clivus, also a favored location for metastases and chondrosarcomas. Hypointense intratumoral septations, Ca++, T2↑↑. 33 •The CT shows some calcifications in this area. The differential diagnosis for this mass would be chordoma or chondrosarcoma •Chordomas tend to occur in the midline, whereas chondrosarcomas tend to occur off the midline. 34

Chondrosarcoma

35  Patient with lung Ca presents with CN VIth palsy due to tumor metastasis.  The normal fatty marrow has been replaced by abnl. tissue. Also lymphomas, myelomas or diffuse bone abnormalities can give this appearance.  Therefore always take a moment to look at the36 clivus! Squamous cell carcinoma Perineural spread

37 37 Hemangiopericytoma

38

ORBITAL ANATOMY

39 ORBITAL COMPARTMENTS

• The orbital space is divided into 4 compartments: 1. Intraconal (ON, orbital fat) 2. Conal (EOMs) 3. Extraconal (LG, preseptal) 4. Globe

40 1/27/2012 40 BONY ORBIT

41 1/27/2012 41

Intraconal: Inflammatory- Infectious  Cellulitis  IOD (Pseudotumor)  Optic neuritis  Sarcoid  TB  Tolosa-Hunt syndrome

42 49 Y WF pain on EM x1wk, ↓ VA: CF 1’ OS DDx: ADEM, MS, NMO, sarcoid, idiopath Etiol: CMV, VZV, mycosis, toxo, syph, tb

43 43 34 y/o ♂ ON-itis OD 6 months later transverse myelitis -Thickened & enhancing ONS

-DDx: cat-scratch, Lyme, sarcoidosis, syphilis, tb, toxo

45 45 Intraconal: Neoplasms  Cavernous hemangioma  Hemangioblastoma (VHL)  Lymphoma (most frequent)/Leukemia  ONG (child w/out NF-1, young adult, elderly)  ONGG (ganglioglioma) may be a/w NF-1  Meningioma (ONSM)  Mets  Rhabdomyosarcoma

 Schwannoma (may be a/w NF-2) 46 10 year-old ♀ with incidentally found ON swelling

47 1/27/2012 47

12/23/2008 Intraneural & Perineural growth ONG Pilocytic astro

48 48 2/22/2008 4/25 6/18

• 75 WF subacute painless visual loss OS and gradual loss of vision OD 6 wks after OS; Labs: CRP 3.31 ESR 22 ACE 36; 49 CSF: cell 0, pr 46, gl 63, MBP+, OCB- IgG index nl 49 75 y/o subacute painless sequential visual loss

50 50 Summary slide of the 3 cases

75 y/o 10 y/o 49 y/o

ONG ONitis 51 ONG 51 A B

C D

: fusiform→axial ; eccentric→extra axial proptosis; Ca++ 52 52 A B C

53 53 Intraconal

3. Metabolic: Krabbe’s dz 4. Vascular: Cavernous hemangioma Dural malformations (ectasia) CCF, IIH Varix (thrombosis)

54 Krabbe dz: 6 month old ♂ with irritability, ON enlargement symmetric T2 ↑ of deep cerebral and peri-dentate nuclei white matter

Shah S et al. Neurology 2012;78:e126-e126

55 ©2012 by Lippincott Williams & Wilkins IIH -Sx: TVO, puls. tinn -MRI/MRV -ONS diameter> 5mm -Correlate with ICP -Tortuous ONs -ONH indents sclera -Empty sella

56 56 Conal: EOM Size & Signal

1. Inflammatory/Infectious Grave’s disease IOD (idiopathic orbital dz: orbital pseudotumor) 2. Tumor- direct extension mets (lymphoma, breast ca, lung ca, carcinoid) rhabdomyosarcoma 57

A Proptosis: from posterior lateral orbital rim to anterior globe > 21mm

B

58 58 48 y/o ♀ with

proptosis & diplopia Acute stage TED Idiopathic orbital disorder A Localized vs generalized -Violates compartments -Involves fat in 75% -Bone distruction -Relative T2 ↓ to muscle -DDx: orbital cellulitis (a/w skin inf or sinusitis) B

IOD 60 60 Localized IOD

Myositis of the levator palpabrae superioris Almekhlafi, M. A. et al. Neurology 2008;71:1202 61 61 ORBITAL CELLULITIS 12 y/o OD pain & fever recurrent sinusitis ↑WBC

62 A -55% of malignant tumors of the orbit -Primary or Secondary -LG & conjuntiva most frequent sites but any B tissue (EOM) can be effected - Leukemia in children -↑T1 (to EOM), ↑T2, marked enhancement C - painless, extra-axial proptosis

63 63 72 y/o ♀ painless diplopia on right gaze Bx: “nl. muscle tissue”

Second opinion?

64 Invasive lobular breast carcinoma

Anti-Estrogen-receptor Atrophic & Dysplastic EOMs

 CN palsy : CN VI, III, IV  NMJ disorder: MG, LEMS  CPEO (chronic progressive external ophthalmoplegia)

66 67 68 CPEO

69 Courtesy Prof. Demer Extraconal

1. Bone lesions: fibrous dysplasia 2. Infection/inflam: granuloma, mucocele 3. Neoplastic: LCH, LG tumors, sinus Ca 4. Periosteal/mesenchymal lesions 5. Vascular: capillary/cavernous hemang., venous lymphatic malformation, varix 6. Trauma: blow-out fractures: inf, medial

70 A B

cranio-orbital: sclerotic-ground glass, cystic degeneration 71 71 A B

C D

Frontoethmoidal: obstruction of the ostium CT: bone remodeling; MRI depends on H2O content 72 A B

C D

73 : encapsulated, solid/myxoid, Ca++,73 mod enh.

Adenoid cystic carcinoma: erodes o. apex, 74 lamina p.: bone erosion, infiltration,Ca74 ++ A B

C D

75 : 80-85% intraconal, lat-to-ON; proliferation followed75 by involution Orbital venous anomaly Intermittent proptosis Eye pain Thombosis: no signal void, if deoxy:↓T1,↓T2 if metHgb: ↑T1,↑T2

76 GLOBE COMPARTMENT • 1. Coloboma, PHPV • 2. staphy, detachments • 3. CMV, Toxocara, VKHS, post. scleritis • 4. Retinoblas, mets, melanoma, choroidal hemangioma (SWS), choroidal osteoma, astrocytic hamartoma (TS) • 5. Retrolental fibroplasia • 6. Coats dz, retinal angiomatosis (VHL)

77 1/27/2012 77

DDx of leukocoria: Retinoblastoma vs. Coats dz. vs. PHPV

DDX of intraocular Ca++: Retinoblastoma vs. ONHD vs. larval granuloma

“Trilateral retinoblastoma”: Retinoblastoma OU & pineal tumor

78 B C

79 Melanoma of the uveal tract: 85 % choroid, 15 % iris or ciliary body; T1▲, T2▼

80 MRI Orbit Protocol

 Noncontrast: Cor T1WI, Cor T2WI-FS, Ax DWI  Contrast: axial & coronal T1WI-FS  STIR paradoxical effect with IV contrast: suppresses tissues with a short T1 (fat, Gad, subacute blood)  SPIR (selective partial IR) selectively inverts signal from fat without disturbing signal from water (SPIR/FLAIR)

81 Szatmáry G. Imaging of the orbit Neurol Clin. 2009 Feb;27(1):251-84 82 DISCLOSURES

Nothing to disclose. Thank you for your attention !

American Society of Neuroimaging 36th Annual Meeting 83