INTRA-OCULAR TUMOURS

INTRA-OCULAR TUMOURS - INTRA-OCULAR TUMOURS Retinoblastoma...

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INTRA-OCULAR TUMOURS
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Retinoblastoma Definition: Retinoblastoma is a proliferation of neural cells that have failed to evolve normally. Retinal cell division continues unchecked and results in development of retinal tumour. Retinoblastoma is found in infants and very young children.
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Epidemiology Disease is rare Frequently tumour is congenital Tumour is exclusively seen in infants and very young children Fellow eye is affected independently, not by metastasis in approximately 1/4 th cases In approximately 10% cases a relative may have suffered from bilateral retinoblastoma
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Epidemiology Presentation – usually 15 months to 24 months of age, most of the cases presents before the age of 3 years Rare after the age of 5 years 30% are bilateral and 70% are unilateral 40% are familial and 60% are non-familial
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Epidemiology Genetic transmission – inheritance is dominant with variable gene penetrance Related to chromosomal abnormality with deletion or mutation of q14 band Mutation of Rb1 gene has been reported
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Pathology Growth consists mainly of small round cells with large nuclei resembling the ceels of nuclear layer of retina. Many of the cells stain poorly indicating necrosis. Rosette shaped arrangement may be present, resembling rods and cones (Flexner Wintersteiner rosettes) In retinoblastoma lesions are usually multiple, with large lesion surrounded by multiple small lesions
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Pathology Microscopically minute deposits are seen scattered in various parts of globe Tumour may grow outwards , separating retina from choroid – Exophytic tumour, condition resembles retinal detachment Or it grow inwards towards vitreous cavity – Endophytic tumour seen as polypoid masses , sometimes haemorrhage on the surface
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Clinical Features SYMPTOMS 1. Child is brought to ophthalmologist with history of yellow /white reflex in pupillary area sometimes called leucocoria or amaurotic cat’s eye 2. Squint, usually convergent , at times divergent 3. Cataract/ Bulging eye/ large eye (buphthalmos)
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Clinical Features Signs
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INTRA-OCULAR TUMOURS - INTRA-OCULAR TUMOURS Retinoblastoma...

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