ABSTRACT

Retinoblastoma (RB) is the commonest ocular malignancy of childhood and is fatal unless treated. In the United Kingdom the incidence of RB is between 1/20000 and 1/23 000 live births, causing about 3% of all childhood cancers. Males and females are equally affected. Based on evaluation by the UK Childhood Cancer Research Group (UKCCRG) it is now possible to provide accurate empiric counselling for affected families. The incidence of bilateral involvement ranges between 25% and 40%. Approximately 10% of all affected children will have a positive family history, though between 8 and 14% of familial cases will only be unilaterally affected. Conversely 3% of unilaterally affected parents will go on to have affected children, of whom more than 90% have bilateral tumours.