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GRAND GRAND ROUNDSROUNDS
Desirée Ong, M.D.Desirée Ong, M.D.
Vanderbilt Eye Institute Vanderbilt Eye Institute
June 28, 2007June 28, 2007
Our PatientOur Patient
CC: “My appearance has changed”CC: “My appearance has changed”
HPI: 57 yo F HPI: 57 yo F Left eye has progressively “pulled Left eye has progressively “pulled
outward” outward” Binocular diplopia x 3 yrsBinocular diplopia x 3 yrs Lashes rub against her sunglassesLashes rub against her sunglasses Students said they did not know who she Students said they did not know who she
was looking at was looking at
HistoryHistory POHX: POHX:
Narrow-angle glaucoma OU s/p LPI OUNarrow-angle glaucoma OU s/p LPI OU Eye muscle surgery x 3 within last two years Eye muscle surgery x 3 within last two years Revision of lower eyelid OS for post-surgical Revision of lower eyelid OS for post-surgical
scarringscarring
PMHX: PMHX: HypothyroidHypothyroid ArthritisArthritis DepressionDepression Concussion x 2Concussion x 2
FHx: Daughter has strabismusFHx: Daughter has strabismus
HistoryHistory Social Hx: Social Hx:
Married with grown childrenMarried with grown children No tobacco or ETOHNo tobacco or ETOH 88thth grade teacher grade teacher
Meds: Meds: WellbutrinWellbutrin SynthroidSynthroid Hormone replacement therapyHormone replacement therapy Alphagan BID OUAlphagan BID OU
Allergies: DarvocetAllergies: Darvocet
ExamExam
BCVA: OD 20/15, OS 20/25-2BCVA: OD 20/15, OS 20/25-2
CVF: full OUCVF: full OU
Ishihara: 15/15 OUIshihara: 15/15 OU
Hertel: 7 mm of proptosis OSHertel: 7 mm of proptosis OS
Pupils: 3Pupils: 32mm OU, no RAPD2mm OU, no RAPD
ExamExam
L/L: quiet OD, inferior symblepharon L/L: quiet OD, inferior symblepharon OSOS
S/C: quiet OUS/C: quiet OU K: quiet OD, PEE OSK: quiet OD, PEE OS A/C: narrow angles OUA/C: narrow angles OU Iris: patent superior LPI OUIris: patent superior LPI OU Lens: 1+ NSC OULens: 1+ NSC OU Ant vitreous: quiet OUAnt vitreous: quiet OU
Differential Diagnosis?Differential Diagnosis?Proptosis with extraocular motility Proptosis with extraocular motility
limitationlimitation
Differential DiagnosisDifferential DiagnosisProptosis with extraocular motility Proptosis with extraocular motility
limitationlimitation Neoplastic Neoplastic
Sphenoid wing meningiomaSphenoid wing meningioma Lacrimal gland tumorLacrimal gland tumor Schwannoma Schwannoma MucoceleMucocele Nasopharyngeal tumorNasopharyngeal tumor Dermoid cystDermoid cyst MetastasisMetastasis Solitary fibrous tumorSolitary fibrous tumor Fibrous histiocytomaFibrous histiocytoma HemangiopericytomaHemangiopericytoma LymphoproliferativeLymphoproliferative NeurofibromaNeurofibroma LeiomyomaLeiomyoma Glioma Glioma
Differential DiagnosisDifferential DiagnosisProptosis with extraocular motility Proptosis with extraocular motility
limitationlimitation AutoimmuneAutoimmune
TED – 60% of all orbital diseaseTED – 60% of all orbital disease Inflammatory Inflammatory
PseudotumorPseudotumor Sarcoid granulomaSarcoid granuloma
Vascular Vascular Cavernous hemangiomaCavernous hemangioma Varix Varix A-V fistulaA-V fistula
InfectiousInfectious Orbital cellulitisOrbital cellulitis Tuberculosis granulomaTuberculosis granuloma
TraumaTrauma Retrobulbar hematomaRetrobulbar hematoma
Imaging: MRIImaging: MRI T1: isointense to brainT1: isointense to brain
T2: hyperintense to brain, T2: hyperintense to brain, isointense to CSFisointense to CSF
Marked enhancement on T1 with Marked enhancement on T1 with gadoliniumgadolinium ““Peripheral ring”Peripheral ring” Cavitary change or heterogeneityCavitary change or heterogeneity
Our PatientOur Patient
Excisional biopsyExcisional biopsyUnderwent anterior orbitotomy with Underwent anterior orbitotomy with
image-guided excision of a well-image-guided excision of a well-encapsulated mass 4/20/07encapsulated mass 4/20/07
Gross PathologyGross Pathology Yellow-tan lesion with Yellow-tan lesion with
varicose vessels on the varicose vessels on the surfacesurface
Cone-shaped > dumbbell Cone-shaped > dumbbell > oval > round> oval > round
True capsule composed True capsule composed of perineuriumof perineurium
Eccentric position within Eccentric position within capsulecapsule
Wt. 3.58 g, dimensions: 3.4 x 2 Wt. 3.58 g, dimensions: 3.4 x 2 x 1.4 cmx 1.4 cm
PathologyPathology
Antoni A pattern: compact Antoni A pattern: compact palisading nucleipalisading nuclei
Verocay bodies: dense whorls
PathologyPathology
Antoni B pattern: ovoid clear cells, loose Antoni B pattern: ovoid clear cells, loose pattern of myxoid stromal degeneration, pattern of myxoid stromal degeneration, cystic spacescystic spaces
PathologyPathology Most common pattern: Most common pattern:
mixed Antoni A and B (47%)mixed Antoni A and B (47%)
No axons present within the No axons present within the substance of the tumor substance of the tumor
Fibrosis and hyalinization Fibrosis and hyalinization around blood vessels, vessel around blood vessels, vessel walls contain xanthoma walls contain xanthoma cellscells
““Ancient” lesions can have Ancient” lesions can have calcification, cystic changescalcification, cystic changes
upload.wikimedia.org/wikipedia/commons/thumb/...
http://www.histopathology-india.net/Schwannoma.htm
‘Ancient’
Luse bodies: cytoplasmic collagen fibrilsLuse bodies: cytoplasmic collagen fibrils
ImmunohistochemistryImmunohistochemistry
S-100 positiveS-100 positive
Vimentin positiveVimentin positive
Leu7 positiveLeu7 positive
Progesterone-receptor positiveProgesterone-receptor positive May account for increased proptosis during May account for increased proptosis during
pregnancypregnancy Possible place for hormonal txPossible place for hormonal tx
http://www.emedicine.com/derm/topic285.htm
Diffuse S-100 positivity
SchwannomaSchwannoma (Neurilemoma)(Neurilemoma)
Benign tumor of Schwann cell Benign tumor of Schwann cell sheaths of peripheral sensory nervessheaths of peripheral sensory nerves
First described by Verocay in 1910First described by Verocay in 1910
Well-defined, slowly progressive and Well-defined, slowly progressive and non-invasive with low malignant non-invasive with low malignant potentialpotential
SchwannomaSchwannoma
No racial predilectionNo racial predilection
Female predominanceFemale predominance
22ndnd to 5 to 5thth decades (mean: 40 yrs) decades (mean: 40 yrs)
18% associated with 18% associated with neurofibromatosisneurofibromatosis
SchwannomaSchwannoma
55% of all peripheral nerve tumors 55% of all peripheral nerve tumors
Predilection for the head/neck, flexor Predilection for the head/neck, flexor surfaces of extremities surfaces of extremities
Solitary except for in NF-1 or 2Solitary except for in NF-1 or 2
Orbital SchwannomaOrbital Schwannoma
1-2% of orbital tumors1-2% of orbital tumors
Extraconal > intraconalExtraconal > intraconal
Superior > medial superior > apexSuperior > medial superior > apex
Orbital SchwannomaOrbital Schwannoma
Most common: sensory branches of CN V1 Most common: sensory branches of CN V1 (supratrochlear/supraorbital)(supratrochlear/supraorbital)
Rarely from motor nerves: CN III (superior Rarely from motor nerves: CN III (superior division, ciliary nerve), CN VIdivision, ciliary nerve), CN VI
Optic nerve never involved since an extension Optic nerve never involved since an extension of the CNSof the CNS
Rarely intramuscular, epibulbar or intraosseusRarely intramuscular, epibulbar or intraosseus
SymptomsSymptoms Initially asymptomaticInitially asymptomatic
DiplopiaDiplopia
HeadacheHeadache
Decreased visionDecreased vision Direct optic nerve compressionDirect optic nerve compression Induced hyperopiaInduced hyperopia
Numbness or pain in distribution of Numbness or pain in distribution of involved nerve – rare unless large tumorinvolved nerve – rare unless large tumor
SignsSigns Proptosis – most commonProptosis – most common
Lid swellingLid swelling
Restricted ocular motilityRestricted ocular motility
Globe displacementGlobe displacement
Afferent pupillary defectAfferent pupillary defect
Choroidal foldsChoroidal folds
Optic atrophy/disc edemaOptic atrophy/disc edema
http://content.lib.utah.edu/cgi-bin/showfile.exe?CISOROOT=/EHSL-WFH&CISOPTR=174
Diagnosis and Diagnosis and ManagementManagement
MRI with/without contrast: imaging MRI with/without contrast: imaging method of choicemethod of choice
Observation vs excision Observation vs excision
Radiation Radiation
Evaluation for NF with bilateral Evaluation for NF with bilateral vestibular schwannomas and age < vestibular schwannomas and age < 30 30
Surgical approach Surgical approach
Governed by location of tumor within the orbitGoverned by location of tumor within the orbit
Complete removal is recommended Complete removal is recommended Aspiration or piecemeal excision Aspiration or piecemeal excision Small risk of recurrence with incomplete removalSmall risk of recurrence with incomplete removal One reported case of recurrence and malignant One reported case of recurrence and malignant
transformation after incomplete excision in a pt transformation after incomplete excision in a pt with neurofibromatosis with neurofibromatosis
May strip tumor from nerve of origin via May strip tumor from nerve of origin via microsurgical techniquemicrosurgical technique
Our Patient at Follow-Up Our Patient at Follow-Up (6/19/07)(6/19/07)
Pt still complains of diplopia in primary gazePt still complains of diplopia in primary gaze Motility:Motility:
Plan: strabismus surgery to improve her Plan: strabismus surgery to improve her diplopiadiplopia
Teaching pointsTeaching points
Early diagnosis and accurate Early diagnosis and accurate evaluation of the extent of an orbital evaluation of the extent of an orbital schwannoma is critical for schwannoma is critical for restoration of vision and preserving restoration of vision and preserving extraocular eye movementsextraocular eye movements
Complete excision, if possible, is Complete excision, if possible, is recommended to prevent recurrence recommended to prevent recurrence and malignant transformation and malignant transformation
ReferencesReferences Chang BY, Moriarty P, Cunniffe G, Barnes C, Kennedy S. Accelerated Chang BY, Moriarty P, Cunniffe G, Barnes C, Kennedy S. Accelerated
growth of a primary orbital schwannoma during pregnancy. Eye. 2003 growth of a primary orbital schwannoma during pregnancy. Eye. 2003 Oct;17(7):839-41.Oct;17(7):839-41.
Cockerham KP, Cockerham GC, Stutzman R et al. The clinical spectrum Cockerham KP, Cockerham GC, Stutzman R et al. The clinical spectrum of schwannomas presenting with visual dysfunction: a clinicopathologic of schwannomas presenting with visual dysfunction: a clinicopathologic study of three cases. Surv Ophthalmol. 1999 Nov-Dec;44(3):226-34. 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 Gunduz K, Shields CL, Gunalp I, Erden E, Shields JA. Correlation of magnetic resonance imaging and pathologic findings. Graefe’s Arch Clin magnetic resonance imaging and pathologic findings. Graefe’s Arch Clin Exp Ophthalmol 2003; 241: 593-597.Exp Ophthalmol 2003; 241: 593-597.
Rawlings NG, Brownstein S, Robinson JW, Jordan DR. Orbital Rawlings NG, Brownstein S, Robinson JW, Jordan DR. Orbital schwannoma: histopathologic correlation with magnetic resonance schwannoma: histopathologic correlation with magnetic resonance imaging. Can J Ophthalmol. 2007 Apr;42(2):326-8. imaging. Can J Ophthalmol. 2007 Apr;42(2):326-8.
Rootman J. Diseases of the Orbit. Philadelphia: J.B. Lippincott Rootman J. Diseases of the Orbit. Philadelphia: J.B. Lippincott Company, 1988: 319-325.Company, 1988: 319-325.
Shields JA, Shields, CL. Atlas of Orbital Tumors. Philadelphia: Shields JA, Shields, CL. Atlas of Orbital Tumors. Philadelphia: Lippincott Williams and Wilkins, 1999: 76-78.Lippincott Williams and Wilkins, 1999: 76-78.
Wang Y, Xiao LH.Wang Y, Xiao LH. Orbital schwannomas: findings from magnetic Orbital schwannomas: findings from magnetic resonance imaging in 62 cases. Eye. 2007 Apr 20; epublication.resonance imaging in 62 cases. Eye. 2007 Apr 20; epublication.