Retinoblastoma

Retinoblastoma is the most commn malignant eye tumor in children, with 95% of cases being diagnosed before 5 years of age. Outcomes vary globally, with extraocular presentation being more common in the low- and middle-income countries resulting in suboptimal survival compared to excellent survival reported in high income countries where an overwhelming majority of tumors present with introcular disease. Two distinct clinical presentations are recognized: (1) bilateral and/or multifocal heritable disease which carries germline mutations of RB1 gene (25% of all cases) and (2) unilateral disease (75% of all cases, 90% of which are non-hereditary). The treatment of retinoblastoma has evolved over the years and is multidisciplinary. Contemporary management strategies are designed to save lives, salvage the globe, preserve vision, and decrease the incidence of second malignancies.