Management of retinoblastoma in children: current status

Abstract

In recent years, there have been dramatic changes in the management of intraocular retinoblastoma. Intraocular retinoblastoma is a highly curable malignancy and current treatments are aimed to preserve vision while reducing the late effects such as treatment-induced secondary malignancies. The advent of intra-arterial chemotherapy changed the treatment paradigm from systemic treatment with chemotherapy to local treatment, and new questions emerged. While intra-arterial chemotherapy achieved encouraging results, only experience from major referral centers is reported, so its indications, advantages and risks are still to be elucidated. Many factors should be considered when choosing the appropriate conservative therapy. When the disease has extended outside the eye, the chances of cure are significantly lower and treatment should be tailored by the presence of pathology risk factors such as invasion of the choroid, the optic nerve, and the sclera. Adjuvant therapy is decided upon this information. Children with overt extraocular disease are treated with higher dose neoadjuvant therapy followed by delayed enucleation and adjuvant therapy.