Download GRAND ROUNDS

GRAND ROUNDS
Desirée Ong, M.D.
Vanderbilt Eye Institute
June 28, 2007
Our Patient
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CC: “My appearance has changed”
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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
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History
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POHX:
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PMHX:
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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
Hypothyroid
Arthritis
Depression
Concussion x 2
FHx: Daughter has strabismus
History
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Social Hx:
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Meds:
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Married with grown children
No tobacco or ETOH
8th grade teacher
Wellbutrin
Synthroid
Hormone replacement therapy
Alphagan BID OU
Allergies: Darvocet
Exam
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BCVA: OD 20/15, OS 20/25-2
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CVF: full OU
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Ishihara: 15/15 OU
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Hertel: 7 mm of proptosis OS
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Pupils: 32mm OU, no RAPD
Motility
Exam
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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
Differential Diagnosis?
Proptosis with extraocular motility limitation
Differential Diagnosis
Proptosis with extraocular motility limitation
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Neoplastic
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Sphenoid wing meningioma
Lacrimal gland tumor
Schwannoma
Mucocele
Nasopharyngeal tumor
Dermoid cyst
Metastasis
Solitary fibrous tumor
Fibrous histiocytoma
Hemangiopericytoma
Lymphoproliferative
Neurofibroma
Leiomyoma
Glioma
Differential Diagnosis
Proptosis with extraocular motility limitation
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Autoimmune
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Inflammatory
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Cavernous hemangioma
Varix
A-V fistula
Infectious
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Pseudotumor
Sarcoid granuloma
Vascular
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TED – 60% of all orbital disease
Orbital cellulitis
Tuberculosis granuloma
Trauma
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Retrobulbar hematoma
What is next?
Imaging: MRI
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T1: isointense to brain
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T2: hyperintense to brain, isointense to CSF
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Marked enhancement on T1 with gadolinium
 “Peripheral
ring”
 Cavitary change or heterogeneity
Our Patient
Excisional biopsy
Underwent anterior orbitotomy with image-guided
excision of a well-encapsulated mass 4/20/07
Gross Pathology
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Yellow-tan lesion with
varicose vessels on the
surface
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Cone-shaped > dumbbell
> oval > round
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True capsule composed of
perineurium
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Eccentric position within
capsule
Wt. 3.58 g, dimensions: 3.4 x 2 x 1.4 cm
Pathology
Verocay bodies: dense whorls
Antoni A pattern: compact palisading nuclei
Pathology
Pathology
Antoni B pattern: ovoid clear cells, loose pattern of myxoid
stromal degeneration, cystic spaces
Pathology
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Most common pattern: mixed
Antoni A and B (47%)
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No axons present within the
substance of the tumor
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Fibrosis and hyalinization around
blood vessels, vessel walls contain
xanthoma cells
Luse bodies: cytoplasmic collagen fibrils
upload.wikimedia.org/wikipedia/commons/thumb/...
“Ancient” lesions can have
calcification, cystic changes
‘Ancient’
http://www.histopathology-india.net/Schwannoma.htm
Immunohistochemistry
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S-100 positive
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Vimentin positive
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Leu7 positive
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Diffuse S-100 positivity
http://www.emedicine.com/derm/topic285.htm
Progesterone-receptor positive
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May account for increased proptosis during pregnancy
Possible place for hormonal tx
Diagnosis
Orbital Schwannoma
Schwannoma
(Neurilemoma)
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Benign tumor of Schwann cell sheaths of
peripheral sensory nerves
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First described by Verocay in 1910
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Well-defined, slowly progressive and noninvasive with low malignant potential
Schwannoma
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No racial predilection
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Female predominance
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2nd to 5th decades (mean: 40 yrs)
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18% associated with neurofibromatosis
Schwannoma
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55% of all peripheral nerve tumors
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Predilection for the head/neck, flexor surfaces of
extremities
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Solitary except for in NF-1 or 2
Orbital Schwannoma
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1-2% of orbital tumors
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Extraconal > intraconal
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Superior > medial superior > apex
Orbital Schwannoma
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Most common: sensory branches of CN V1
(supratrochlear/supraorbital)
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Rarely from motor nerves: CN III (superior division,
ciliary nerve), CN VI
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Optic nerve never involved since an extension of the CNS
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Rarely intramuscular, epibulbar or intraosseus
Symptoms
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Initially asymptomatic
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Diplopia
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Headache
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Decreased vision
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Direct optic nerve compression
Induced hyperopia
Numbness or pain in distribution of involved nerve –
rare unless large tumor
Signs
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Proptosis – most common
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Lid swelling
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Restricted ocular motility
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Globe displacement
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Afferent pupillary defect
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Choroidal folds
http://content.lib.utah.edu/cgi-bin/showfile.exe?CISOROOT=/EHSL-WFH&CISOPTR=174
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Optic atrophy/disc edema
Diagnosis and Management
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MRI with/without contrast: imaging method of
choice
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Observation vs excision
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Radiation
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Evaluation for NF with bilateral vestibular
schwannomas and age < 30
Surgical approach
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Governed by location of tumor within the orbit
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Complete removal is recommended
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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
Our Patient at Follow-Up (6/19/07)
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Pt still complains of diplopia in primary gaze
Motility:
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Plan: strabismus surgery to improve her diplopia
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Teaching points
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Early diagnosis and accurate evaluation of the
extent of an orbital schwannoma is critical for
restoration of vision and preserving extraocular
eye movements
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Complete excision, if possible, is recommended
to prevent recurrence and malignant
transformation
References
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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: 319325.
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.