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28-07-2016 | Ophthalmologic cancers | Article

37. Retinoblastoma

Authors: Carlos A. Medina, MD, Priyanka Kumar, MD, Arun D. Singh, MD

Publisher: Springer New York

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Abstract

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.
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