ABSTRACT

Retinoblastoma is a rare tumor of both scientific and public health importance. Scientifically, this ocular condition provides unique opportunities for the evaluation of specific genetic abnormalities associated with tumorigenesis [1]. These data help elucidate the processes whereby both ocular and systemic tumors may develop [2,3]. From a public health perspective, the importance of retinoblastoma transcends even the tragedy associated with the death of a child. Although current treatments have resulted in survival better than 90%, these children and society have the burden of coping with long-term visual and systemic morbidity. More recently, systemic chemotherapy coupled with local intraocular therapy have become accepted treatment to avoid the use of external-beam radiation, which has been associated with disfigurement of the face, poor visual results, and a 35% risk of secondary cancers during a 30-year period [4-9]. Treatment of large tumors, especially those with vitreous seeding, remains difficult, as they have responded poorly to both the chemotherapy and the traditional treatment of radiation. Such eyes have often required enucleation. Once the tumor extends outside the eye, the prognosis is dismal.