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This is leucocoria, or "white pupil" caused by the presence of a mass lesion in
the eye--a retinoblastoma. This is the most common intraocular neoplasm of childhood.
INTRAOCULAR TUMOURS
DR K.S.RATNAKAR
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INTRAOCULAR NEOPLASTIC LESIONS
• Dr K.S.Ratnakar
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RETINO BLASTOMA –CLINICAL PRESENTATION
• CAT’S EYE – 33 (42.3%)
• PROPTOSIS – 28 (35.9%)
• SQUINT – 6 (7.7%)
• OTHERS – 11 (14.1%)
• UNILATERAL – 63 (80.8%)
• BILATERAL – 15 (19.2%)
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RETINOBLASTOMA –AGE & SEX DISTRIBUTION
AGE MALE FEMALE TOTAL %
0-2 8 4 12 15.38
2-4 20 13 13 42.30
4-6 16 6 22 28.20
6-8 5 2 7 8.97
8-10 3 1 4 5.12
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Retinoblastoma is the classic example of the neoplasm arising from the "two hit" genetic defect. If the patient inherits one bad tumor suppressor gene (the Rb gene on chromosome 13), either by a point mutation or by deletion of the locus q14 on chromosome 13 as pictured here, then the other is typically lostin childhood and a retinoblastoma develops.
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RETINOBLASTOMA -HISTOLOGY
•OOVAL TO SPINDLE CELLS WITH DARK NUCLEI AND SCANT CYTOPLASM•RROSETTE-FW, HW•PPSEUDOROSETTES•NNECROSIS•CCALCIFICATION•VVALCULAR BASOPHILIA•OTHER FEATURES OF DIFFERENTIATION
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CLINICAL CHARACTERISTICS OF
RETINOMAS(RETINOCYTOMAS)
•C-COMPARATIVELY SMALL, HOMOGENOUS, TRANSLUCENT, GRAY, SLIGHTLY ELEVATED PLACOID MASS WITH FUNCTIONAL RETINAL BLOOD VESSELS LOOPING INTO THE MASS.•O-OPAQUE, WHITE CALCIFIED FLECKS HAVE APPEARANCE OF COTTAGE CHEESE•P-PROLIFERATION AND MIGRATION OF REINAL PIGMENT EPITHELIUM IN AREAS UNDERLYING OR ADJACENT OT THE TUMORS
•F-FUNCTIONAL EYE WITH CLEAR MEDIA AND NO RETINAL DETACHMENT.
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HISTOLOGIC COMPARISON
Larger and more hyperchromaticnucleiScanty cytoplasm and intercellular matrixNumerous mitotic figures
Necrosis highly characteristicCalcification in areas of necrosisDifferentiation into Flexner-Wintersteiner rosettesCytologically malignant
Smaller and less hyperchromaticnucleiAbundant cytoplasm and intercellular matrixMitotic figures absent or very rareNecrosis typically absent
Calcification in non-necrotic tumorDifferentiation into fleurettes
Cytologically benign
RETINOBLASTOMAS RETINOCYTOMAS
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Classification of retinoblastoma into undifferentiated and differentiated types neither conveys histogenesis nor prognosis.- Earlier view – RB with rosettes better
prognosis.- Differentiated RB with FW or HW rosettee
don’t offer better prognosis (Taktikos, 1966, TSO et al, 1970).
RETINOBLASTOMAThis watermark does not appear in the registered version - http://www.clicktoconvert.com
RETINOBLASTOMA PROGNOSIS
•DDIFFERENTIATION •CCHOROIDAL INVASION•OOPTIC NERVE INVOLVEMENT (AXIAL/SUBARACHNOIDAL)•>5% SMALL TUMOURS – CHOROIDAL INVASION•PPOSTERIOR POLE TUMOURS –EARLY INVASION.
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RETINOBLASTOMA
•HHistologically multiple foci of tumor appreciated on serial section study•NNecrosis common in exuberant tumours•VVascular basophilia (Feulgenophilia)•DDifferentiation like ganglion cells, axis cylinders orspongioblastic astrocytes ocdasionally observed.
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COAT’S DISEASE
•UUSUALLY UNILATERAL •TTWO-THIRDS MALES•CCAN AFFECT 18 MONTHS –18 YEARS AGE
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PATHOGENESIS OF COAT’S DISEASE
ALTERED ENDOTHELIL PERMEABILITY
TELANGIECTASIA MURAL PLASMA-THICKENING EXUDATION
EXUDATIVERETINALDETACHMENT
ALBUMINOUS FOAM CELLS CHOLES-FLUID GHOST CELLS TEROLCLEFTS
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MEDULLOEPITHELIOMA
•M-Malignant tumor : 10% mortality-Bad prognostic factors:-extrascleral extension -optic nerve involvement
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MEDULLOEPITHELIOMA
•CCongenital tumor, usually unilateral•AArises from primitivemedullary epithelium in iris,ciliary body, retina, optic, disc, optic nerve associated with PHPV
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MALIGNANT MELANOMA
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MALIGNANT MELANOMA
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Where do melanomas arise from ?
STROMAL MELANOCYTESTumour associated with melanosis
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Diffuse malignant melanomas
•DDifficult to detect•OOften treated for glaucoma,uveitis or detachment•AAlmost always mixed cells type•1>3% show extrascleralextension•5 5year mortality – 73%
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Malignant melanoma of choroid Modes of Spread
•I-Intraocular and Episcleral•O-Orbital•D-Distant metastases
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EFFECTS OF THE MELANOMA ON INTRAOCULAR STRUCTURES
•R-Retina and RPE Retinal Detachment Cystoiddegeneration-Lipofuscin deposits-Subretinal Neovascularization-RPE metaplasia and atrophy. Drusen•C-Choroid – Choriocapillaris compression-Glaucoma – melanomalytic glaucoma in necrotic tumors. Peripheral & diffuse tumors.
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MALIGNANT MELANOMACLASSIFICATION
Ú SPINDLE CELL NAEVUS
ÚSPINDLE CELL MELANOMA
ÚMIXED CELL MELANOMA
ÚEPITHELIOID CELL MELANOMA
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MALIGNANT MELANOMAHISTOLOGY
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MALIGNANT MELANOMAHISTOLOGY
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MALIGNANT MELANOMAHISTOLOGY – UNUSUAL VARIANTS
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MALIGNANT MELANOMA CILIARY BODY
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MALIGNANT MELANOMA - IRIS
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MELANOCYTOMA
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LYMPHOMA
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HAEMANGIOMA -CHOROID
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PHTHISIS BULBIWITH RPEHYERPLASIA&OSSIFICATION
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