INTRAOCULAR - Directorate of Medical Educationdme.ap.nic.in/Intraocular.pdf · This is leucocoria,...

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

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%)

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

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.

RETINOBLASTOMA -HISTOLOGY

•OOVAL TO SPINDLE CELLS WITH DARK NUCLEI AND SCANT CYTOPLASM•RROSETTE-FW, HW•PPSEUDOROSETTES•NNECROSIS•CCALCIFICATION•VVALCULAR BASOPHILIA•OTHER FEATURES OF DIFFERENTIATION

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.

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

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).

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RETINOBLASTOMA PROGNOSIS

•DDIFFERENTIATION •CCHOROIDAL INVASION•OOPTIC NERVE INVOLVEMENT (AXIAL/SUBARACHNOIDAL)•>5% SMALL TUMOURS – CHOROIDAL INVASION•PPOSTERIOR POLE TUMOURS –EARLY INVASION.

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.

COAT’S DISEASE

•UUSUALLY UNILATERAL •TTWO-THIRDS MALES•CCAN AFFECT 18 MONTHS –18 YEARS AGE

PATHOGENESIS OF COAT’S DISEASE

ALTERED ENDOTHELIL PERMEABILITY

TELANGIECTASIA MURAL PLASMA-THICKENING EXUDATION

EXUDATIVERETINALDETACHMENT

ALBUMINOUS FOAM CELLS CHOLES-FLUID GHOST CELLS TEROLCLEFTS

MEDULLOEPITHELIOMA

•M-Malignant tumor : 10% mortality-Bad prognostic factors:-extrascleral extension -optic nerve involvement

MEDULLOEPITHELIOMA

•CCongenital tumor, usually unilateral•AArises from primitivemedullary epithelium in iris,ciliary body, retina, optic, disc, optic nerve associated with PHPV

MALIGNANT MELANOMA

Where do melanomas arise from ?

STROMAL MELANOCYTESTumour associated with melanosis

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%

Malignant melanoma of choroid Modes of Spread

•I-Intraocular and Episcleral•O-Orbital•D-Distant metastases

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.

MALIGNANT MELANOMACLASSIFICATION

Ú SPINDLE CELL NAEVUS

ÚSPINDLE CELL MELANOMA

ÚMIXED CELL MELANOMA

ÚEPITHELIOID CELL MELANOMA

MALIGNANT MELANOMAHISTOLOGY

MALIGNANT MELANOMAHISTOLOGY – UNUSUAL VARIANTS

MALIGNANT MELANOMA CILIARY BODY

MALIGNANT MELANOMA - IRIS

MELANOCYTOMA

LYMPHOMA

HAEMANGIOMA -CHOROID

PHTHISIS BULBIWITH RPEHYERPLASIA&OSSIFICATION

INTRAOCULAR - Directorate of Medical Educationdme.ap.nic.in/Intraocular.pdf · This is leucocoria,...

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