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Purpose of review: To provide an update on current management of retinoblastoma

Recent findings: The current major treatment strategies for retinoblastoma involve enucleation, intravenous chemoreduction, and intra-arterial chemotherapy. Enucleation is reserved for eyes with extensive retinoblastoma, in which there is no hope for useful vision. Newer implants following enucleation allow excellent cosmetic and motility outcome. Intravenous chemoreduction has been popular for nearly two decades and continues to provide favorable tumor control for most eyes classified as groups A, B, or C and some D eyes, using the International Classification of Retinoblastoma. Using chemoreduction, there has been an apparent reduction in the incidence of pinealoblastoma in children with germline mutation retinoblastoma. Intra-arterial chemotherapy involves single-agent injection into the ophthalmic artery under careful neurointerventional guidance. This recently popularized therapy can be useful for eyes that fail standard treatments or for some eyes as a primary treatment. Short-term results are favorable but longer follow-up is warranted.

Summary: Progress has been made in the past few years in the management of retinoblastoma with better enucleation implants, chemoreduction-prevention of pinealoblastoma and excellent tumor control, and we face the recently popularized modality of intra-arterial chemotherapy with caution and hope.

(C) 2010 Lippincott Williams & Wilkins, Inc.