Clinicopathologic Features of Retinoblastoma After Primary Chemoreduction

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Abstract
RETINOBLASTOMA is the most common intraocular tumor in childhood, occurring in 1 of 17000 to 24000 live births,1-3 independent of race and sex.4 With modern methods of treatment, the survival rate is more than 90%, while untreated, retinoblastoma is always fatal.5 Retinoblastoma may occur as a hereditary or nonhereditary tumor. Tumors in nonhereditary retinoblastoma are typically solitary and unilateral with no family history and no detectable chromosomal abnormalities. About 40% of retinoblastoma are caused by a germ line mutation and include the cases with a positive family history.6 Most children with hereditary mutations develop multifocal retinoblastoma in both eyes. As many as 15% of sporadic unilateral retinoblastomas may be hereditary.7