Abstract
The management of retinoblastoma has evolved remarkably for the past century, and lately there is a drift towards focal conservative treatments. The challenge remains, however, in maintaining the eye and vision. Tumor reduction by diverse first-line chemotherapy protocols followed by local treatments like cryotherapy, laser photocoagulation, thermotherapy, and plaque radiotherapy is now accepted as treatment strategy for intraocular retinoblastoma with the goal of avoiding external beam radiotherapy (EBRT) or enucleation. However in most eyes with large tumors with diffuse vitreous and subretinal seeds, the EBRT or enucleation is eventually required. Chemoprophylaxis is necessary for preventing metastases in tumors with high-risk pathology features (tumor extending beyond lamina cribrosa of the optic nerve, tumor extending till the surgical margin of the optic nerve, massive choroidal invasion, scleral involvement or anterior segment involvement). Intensified chemotherapy with autologous stem cell rescue appears effective for patients with metastatic retinoblastoma. Recently, ophthalmic intra-arterial infusion with melphalan is proved as an excellent globe conserving treatment option in advanced retinoblastoma cases with minimal systemic side effects. Progress in the clinical recognition and management of retinoblastoma has led to high survival rates.
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Shah, P.K., Aurora, S., Narendran, V., Kalpana, N. (2012). Pediatric Intraocular Retinoblastoma: Treatment. In: Hayat, M. (eds) Pediatric Cancer, Volume 3. Pediatric Cancer, vol 3. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-4528-5_21
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