Retinoblastoma

-primary intra-ocular malignancy of children

- cell of origin is neuronal

- 1/3 of cases occur in patients who inherit a germline mutation of one RB (retinoblastoma gene) allele

- inherited retinoblastoma is often bilateral as well as trilateral, with a pinealoblastoma

Treatment

-          Chemotherapy initially

-          Followed by laser treatment or cryopexy

-          This tumor tends to spread to the brain and bone marrow

-          Poor prognostic factors include extra-ocular extension, invasion along optic nerve, choroidal invasion

-          Pre-malignant lesion is the retinocytoma, also known as the retinoma