Based on personal experience with 324 children with retinoblastoma who were managed on the Ocular Oncology Service at Wills Eye Hospital between 1974 and 1988, the authors review their current management of this tumor, emphasize common misconceptions in management, and describe the recent research related to the genetics of retinoblastoma. It is pointed out that the optic nerve should not be clamped at the time of enucleation. Photocoagulation should only be used to treat small tumors strictly confined to the retina and it is contraindicated if there is vitreal or choroidal invasion. The tumor should not be directly photocoagulated but rather surrounded by photocoagulation to destroy its blood supply. Cryotherapy should generally be applied to small peripheral tumors and should not be attempted if there is evidence of vitreous seeding of the tumor. External beam irradiation should employ an anterior as well as a lateral portal. Carefully selected radioactive plaque therapy, when it can be used, has distinct advantages over external beam radiotherapy.
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