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 This watermark does not appear in the registered version - http://www.clicktoconvert.com
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INTRAOCULAR - Directorate of Medical Educationdme.ap.nic.in/Intraocular.pdf · This is leucocoria, or "white pupil" caused by the presence of a mass lesion in the eye--a retinoblastoma.
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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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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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•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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•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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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
•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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•CCongenital tumor, usually unilateral•AArises from primitivemedullary epithelium in iris,ciliary body, retina, optic, disc, optic nerve associated with PHPV
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•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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