Update on Sellar & Orbital Imaging

Transcription

1 Update on Sellar & Orbital Imaging Gabriella Szatmáry, MD, PhD Director of Neuro-Ophthalmology Neuroimager Hattiesburg Clinic, PA American Society of Neuroimaging 36 th Annual Meeting

2 01/19/2013 2

3 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

4 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

5 9/2008 2/2008 4/2008 Pituitary apoplexy 86 y/o VA, anorexia

6 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

7 Pituitary abscess 28-year-old man with febrile cephalalgia 7

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

9 Infundibular lesions 9

10 13 y/o VA SITA Standard 24-2 O.S. O.D.

11 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: , March

12 LANGERHANS CELL HISTIOCYTOSIS X 24 y/o with DI Bx of scalp and groin lesion T12 & pit mets Proliferative do. of Langerhan s cells

13 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 neurohypophysis. Benign lesions, also called pituicytoma or choristoma. (Courtesy of L. Mechtler) 13

14 Pituitary protocol Image Plane Sequence Slice / Gap FOV Sagittal T1 3mm / 0mm 12 x 12 Coronal Coronal *Coronal (pituitary gland) T1 T2 Dynamic TSE/FSE T1+C 3mm / 0mm 12 x 12 3mm / 0mm 12 x 12 3mm / 0mm 12 x 12 Sagittal T1+C 3mm / 0mm 12 x 12 Coronal T1+C Fat Sat 3mm / 0mm 12 x 12 Axial whole brain T1+C Fat Sat x 12

15 Extrasellar: Suprasellar Craniopharyngioma Tuber cinereum hamartoma Arachnoid cyst Dermoid, Epidermoid Inflammation-infection Vascular 15

16 Craniopharyngioma epithelial: adamantinous or squamous-papillary Sartoretti-Schefer et al. Am J Neuroradiol 18:77 87, 16

17 Germinoma ma 80-90% along IIIrd ventricle 5-10% basal ggl Bifocal germinoma: pineal + suprasellar DWI restriction: high cellularity Intense homogeneous Gad+ ± CSF seeding, ± brain invasion 17

18 Hypothalamic/Optic Chiasm Gliomas 15-30% associated with NF-1 18

19 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

20 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

21 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

22 Dermoid Cyst <0.5% if primary intracranial tumors Ectopic cyst containing dermal elements Less common than epidermoid Rupture causes significant morbidity and mortality 22

23 EPIDERMOID CYST -1% of all IC tumors -At CPA, parasellar, 4 th ventricular regions -Fast-FLAIR & DWI best sequences 23

24 45 Y/O WOMAN WITH CONFUSION Neurosarcoidosis Solitary or multifocal CNS masses, dural plaque, parenchymal (non-caseating granulomas) 24

25 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

26 Extrasellar: Parasellar Meningioma Schwannoma THS CCF Cavernous hemang Thrombosis Lemierre syn 26

27 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

28 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 hours of initiation of steroid therapy Exclusion of other etiologies by appropriate investigation, including neuroimaging. 28

29 Dx? B 29 29

30 40 y/o galactorrhea, PRL:

31 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

32 Extrasellar: Infrasellar 32

33 Chordoma Chordomas are the most common lesions of the clivus, also a favored location for metastases and chondrosarcomas. Hypointense intratumoral septations, Ca++, T2. 33

34 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

35 Chondrosarcoma 35

36 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 the clivus! 36

37 Squamous cell carcinoma Perineural spread 37 37

38 Hemangiopericytoma 38

39 ORBITAL ANATOMY 39

40 ORBITAL COMPARTMENTS The orbital space is divided into 4 compartments: 1. Intraconal (ON, orbital fat) 2. Conal (EOMs) 3. Extraconal (LG, preseptal) 4. Globe 1/27/

41 BONY ORBIT 1/27/

42 Intraconal: Inflammatory- Infectious Cellulitis IOD (Pseudotumor) Optic neuritis Sarcoid TB Tolosa-Hunt syndrome 42

43 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

44 34 y/o ON-itis OD 6 months later transverse myelitis

45 -Thickened & enhancing ONS -DDx: cat-scratch, Lyme, sarcoidosis, syphilis, tb, toxo 45 45

46 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

47 10 year-old with incidentally found ON swelling 1/27/

48 12/23/2008 Intraneural & Perineural growth ONG Pilocytic astro 48 48

49 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; CSF: cell 0, pr 46, gl 63, MBP+, OCB- IgG index nl 49 49

50 75 y/o subacute painless sequential visual loss 50 50

51 Summary slide of the 3 cases 75 y/o 10 y/o 49 y/o ONG ONG ONitis 51 51

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

53 A B C 53 53

54 Intraconal 3. Metabolic: Krabbe s dz 4. Vascular: Cavernous hemangioma Dural malformations (ectasia) CCF, IIH Varix (thrombosis) 54

55 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-e by Lippincott Williams & Wilkins 55

56 IIH -Sx: TVO, puls. tinn -MRI/MRV -ONS diameter> 5mm -Correlate with ICP -Tortuous ONs -ONH indents sclera -Empty sella 56 56

57 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

58 A Proptosis: from posterior lateral orbital rim to anterior globe > 21mm B 58 58

59 48 y/o with proptosis & diplopia Acute stage TED

60 A B Idiopathic orbital disorder Localized vs generalized -Violates compartments -Involves fat in 75% -Bone distruction -Relative T2 to muscle -DDx: orbital cellulitis (a/w skin inf or sinusitis) IOD 60 60

61 Localized IOD Myositis of the levator palpabrae superioris Almekhlafi, M. A. et al. Neurology 2008;71:

62 ORBITAL CELLULITIS 12 y/o OD pain & fever recurrent sinusitis WBC 62

63 A B C -55% of malignant tumors of the orbit -Primary or Secondary -LG & conjuntiva most frequent sites but any tissue (EOM) can be effected - Leukemia in children - T1 (to EOM), T2, marked enhancement - painless, extra-axial proptosis 63 63

64 72 y/o painless diplopia on right gaze Bx: nl. muscle tissue Second opinion? 64

65 Invasive lobular breast carcinoma Anti-Estrogen-receptor

66 Atrophic & Dysplastic EOMs CN palsy : CN VI, III, IV NMJ disorder: MG, LEMS CPEO (chronic progressive external ophthalmoplegia) 66

67 67

68 68

69 CPEO Courtesy Prof. Demer 69

70 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

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

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

73 A B C D : encapsulated, solid/myxoid, Ca++, 73 mod enh. 73

74 Adenoid cystic carcinoma: erodes o. apex, lamina p.: bone erosion, infiltration,ca

75 A B C D : 80-85% intraconal, lat-to-on; proliferation followed 75 by involution 75

76 Orbital venous anomaly Intermittent proptosis Eye pain Thombosis: no signal void, if deoxy: T1, T2 if methgb: T1, T2 76

77 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) 1/27/

78 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

79 B C 79

80 Melanoma of the uveal tract: 85 % choroid, 15 % iris or ciliary body; T1, T2 80

81 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

82 Szatmáry G. Imaging of the orbit Neurol Clin Feb;27(1):

83 DISCLOSURES Nothing to disclose. Thank you for your attention! American Society of Neuroimaging 36 th Annual Meeting 83