Retinoblastoma: Therapeutic Options

Abstract

Over the past few years, there has been increasing interest in local or regional delivery of chemotherapeutic agents in an attempt to reduce the morbidity of less selective modalities. In this chapter, we summarize current management approaches for intraocular retinoblastoma (intravenous, intra-arterial, periocular, and intravitreal chemotherapy), focal modalities (cryotherapy, transpupillary thermotherapy, and brachytherapy) and local therapies (external radiation therapy and enucleation). The international classification of intraocular retinoblastoma offers guidance to clinicians in deciding when and how to manage intraocular retinoblastoma. Most oncology centers will attempt to save Group A-C eyes, even if central vision is poor and the patient has unilateral disease, because of the high success rates achieved with current approaches (80-100%). Conversely, the likelihood of salvaging Group D eyes with chemotherapy alone is about 50%, which creates a dilemma for unilateral patients. If the visual potential is poor (macular tumor), enucleation for unilateral Group D disease can be considered. In general, Group E eyes should be enucleated. Genetic testing is integral to the overall management of retinoblastoma patients.