MD Carlos A. Medina, MD Priyanka Kumar, MD Arun D. Singh
Springer New York
This chapter considers general aspects and clinical features of retinoblastoma, an intraocular malignancy of primitive neuroendocrine origin that primarily affects young children. In the United States, the age-adjusted incidence of retinoblastoma is approximately 11.8 per million children aged 0–4 years, which represents 6.1 % of all childhood cancers under age 5 years. Depending on the classification of the tumor, globe-sparing treatment may be advised and should be performed by a clinician with experience in the treatment of retinoblastoma. A number of treatment options are available including local or systemic chemotherapy, chemoreduction, photocoagulation, thermotherapy, cryotherapy, brachytherapy, and enucleation. Treatment selection is complex and should be personalized to every case through a multidisciplinary approach. Genetic counseling is integral to the management of retinoblastoma patients.