Current management strategies for intraocular retinoblastoma.

Overview

Survival rates for retinoblastoma patients have increased dramatically over the last century, with documented 5-year survival figures reaching 87-99% in developed countries. During the last decade, there has been a dramatic paradigm shift in the treatment approach for intraocular retinoblastoma, emphasising chemoreduction protocols and minimising the use of external beam radiation. The recognition of the increased risk for second non-ocular cancers with external beam radiation contributed to the growing emergence of chemotherapy. Although chemoreduction protocols vary slightly between institutions, many centres are currently treating intraocular retinoblastoma with carboplatin, vincristine and etoposide as a three-drug regimen given in two to six cycles. Clinical studies have demonstrated that systemic chemotherapy must be combined with other modalities, such as laser treatment and cryotherapy, for adequate tumour control and many eyes with advanced intraocular disease require salvage therapy with radiation or enucleation. Therefore, modern centres treating retinoblastoma continue to manage patients with a variety of modalities, individualising the therapy according to the patient's presentation and clinical course.