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GRAND ROUNDS

GRAND ROUNDS. Desirée Ong, M.D. Vanderbilt Eye Institute June 28, 2007. Our Patient. CC: “My appearance has changed” HPI: 57 yo F Left eye has progressively “pulled outward” Binocular diplopia x 3 yrs Lashes rub against her sunglasses

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GRAND ROUNDS

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  1. GRAND ROUNDS Desirée Ong, M.D. Vanderbilt Eye Institute June 28, 2007

  2. Our Patient • CC: “My appearance has changed” • HPI: 57 yo F • Left eye has progressively “pulled outward” • Binocular diplopia x 3 yrs • Lashes rub against her sunglasses • Students said they did not know who she was looking at

  3. History • POHX: • Narrow-angle glaucoma OU s/p LPI OU • Eye muscle surgery x 3 within last two years • Revision of lower eyelid OS for post-surgical scarring • PMHX: • Hypothyroid • Arthritis • Depression • Concussion x 2 • FHx: Daughter has strabismus

  4. History • Social Hx: • Married with grown children • No tobacco or ETOH • 8th grade teacher • Meds: • Wellbutrin • Synthroid • Hormone replacement therapy • Alphagan BID OU • Allergies: Darvocet

  5. Exam • BCVA: OD 20/15, OS 20/25-2 • CVF: full OU • Ishihara: 15/15 OU • Hertel: 7 mm of proptosis OS • Pupils: 32mm OU, no RAPD

  6. Motility

  7. Exam • L/L: quiet OD, inferior symblepharon OS • S/C: quiet OU • K: quiet OD, PEE OS • A/C: narrow angles OU • Iris: patent superior LPI OU • Lens: 1+ NSC OU • Ant vitreous: quiet OU

  8. Differential Diagnosis?Proptosis with extraocular motility limitation

  9. Differential DiagnosisProptosis with extraocular motility limitation • Neoplastic • Sphenoid wing meningioma • Lacrimal gland tumor • Schwannoma • Mucocele • Nasopharyngeal tumor • Dermoid cyst • Metastasis • Solitary fibrous tumor • Fibrous histiocytoma • Hemangiopericytoma • Lymphoproliferative • Neurofibroma • Leiomyoma • Glioma

  10. Differential DiagnosisProptosis with extraocular motility limitation • Autoimmune • TED – 60% of all orbital disease • Inflammatory • Pseudotumor • Sarcoid granuloma • Vascular • Cavernous hemangioma • Varix • A-V fistula • Infectious • Orbital cellulitis • Tuberculosis granuloma • Trauma • Retrobulbar hematoma

  11. What is next?

  12. Imaging: MRI • T1: isointense to brain • T2: hyperintense to brain, isointense to CSF • Marked enhancement on T1 with gadolinium • “Peripheral ring” • Cavitary change or heterogeneity

  13. Our Patient Excisional biopsy Underwent anterior orbitotomy with image-guided excision of a well-encapsulated mass 4/20/07

  14. Gross Pathology • Yellow-tan lesion with varicose vessels on the surface • Cone-shaped > dumbbell > oval > round • True capsule composed of perineurium • Eccentric position within capsule Wt. 3.58 g, dimensions: 3.4 x 2 x 1.4 cm

  15. Pathology Verocay bodies: dense whorls • Antoni A pattern: compact palisading nuclei

  16. Pathology

  17. Pathology Antoni B pattern: ovoid clear cells, loose pattern of myxoid stromal degeneration, cystic spaces

  18. Pathology • Most common pattern: mixed Antoni A and B (47%) • No axons present within the substance of the tumor • Fibrosis and hyalinization around blood vessels, vessel walls contain xanthoma cells • “Ancient” lesions can have calcification, cystic changes Luse bodies: cytoplasmic collagen fibrils upload.wikimedia.org/wikipedia/commons/thumb/... ‘Ancient’ http://www.histopathology-india.net/Schwannoma.htm

  19. Immunohistochemistry Diffuse S-100 positivity • S-100 positive • Vimentin positive • Leu7 positive • Progesterone-receptor positive • May account for increased proptosis during pregnancy • Possible place for hormonal tx http://www.emedicine.com/derm/topic285.htm

  20. Diagnosis Orbital Schwannoma

  21. Schwannoma(Neurilemoma) • Benign tumor of Schwann cell sheaths of peripheral sensory nerves • First described by Verocay in 1910 • Well-defined, slowly progressive and non-invasive with low malignant potential

  22. Schwannoma • No racial predilection • Female predominance • 2nd to 5th decades (mean: 40 yrs) • 18% associated with neurofibromatosis

  23. Schwannoma • 55% of all peripheral nerve tumors • Predilection for the head/neck, flexor surfaces of extremities • Solitary except for in NF-1 or 2

  24. Orbital Schwannoma • 1-2% of orbital tumors • Extraconal > intraconal • Superior > medial superior > apex

  25. Orbital Schwannoma • Most common: sensory branches of CN V1 (supratrochlear/supraorbital) • Rarely from motor nerves: CN III (superior division, ciliary nerve), CN VI • Optic nerve never involved since an extension of the CNS • Rarely intramuscular, epibulbar or intraosseus

  26. Symptoms • Initially asymptomatic • Diplopia • Headache • Decreased vision • Direct optic nerve compression • Induced hyperopia • Numbness or pain in distribution of involved nerve – rare unless large tumor

  27. Signs • Proptosis – most common • Lid swelling • Restricted ocular motility • Globe displacement • Afferent pupillary defect • Choroidal folds • Optic atrophy/disc edema http://content.lib.utah.edu/cgi-bin/showfile.exe?CISOROOT=/EHSL-WFH&CISOPTR=174

  28. Diagnosis and Management • MRI with/without contrast: imaging method of choice • Observation vs excision • Radiation • Evaluation for NF with bilateral vestibular schwannomas and age < 30

  29. Surgical approach • Governed by location of tumor within the orbit • Complete removal is recommended • Aspiration or piecemeal excision • Small risk of recurrence with incomplete removal • One reported case of recurrence and malignant transformation after incomplete excision in a pt with neurofibromatosis • May strip tumor from nerve of origin via microsurgical technique

  30. Our Patient at Follow-Up (6/19/07) • Pt still complains of diplopia in primary gaze • Motility: • Plan: strabismus surgery to improve her diplopia

  31. Teaching points • Early diagnosis and accurate evaluation of the extent of an orbital schwannoma is critical for restoration of vision and preserving extraocular eye movements • Complete excision, if possible, is recommended to prevent recurrence and malignant transformation

  32. References • Chang BY, Moriarty P, Cunniffe G, Barnes C, Kennedy S. Accelerated growth of a primary orbital schwannoma during pregnancy. Eye. 2003 Oct;17(7):839-41. • Cockerham KP, Cockerham GC, Stutzman R et al. The clinical spectrum of schwannomas presenting with visual dysfunction: a clinicopathologic study of three cases. Surv Ophthalmol. 1999 Nov-Dec;44(3):226-34. • Gunduz K, Shields CL, Gunalp I, Erden E, Shields JA. Correlation of magnetic resonance imaging and pathologic findings. Graefe’s Arch Clin Exp Ophthalmol 2003; 241: 593-597. • Rawlings NG, Brownstein S, Robinson JW, Jordan DR. Orbital schwannoma: histopathologic correlation with magnetic resonance imaging. Can J Ophthalmol. 2007 Apr;42(2):326-8. • Rootman J. Diseases of the Orbit. Philadelphia: J.B. Lippincott Company, 1988: 319-325. • Shields JA, Shields, CL. Atlas of Orbital Tumors. Philadelphia: Lippincott Williams and Wilkins, 1999: 76-78. • Wang Y, Xiao LH.Orbital schwannomas: findings from magnetic resonance imaging in 62 cases. Eye. 2007 Apr 20; epublication.

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