Management of high-risk retinoblastoma

Abstract

While intraocular retinoblastoma is highly curable and current treatments are aimed to preserve vision, once the disease has extended outside the eye, the chances of cure are significantly lower. Thus, extraocular dissemination is the leading cause of death caused by retinoblastoma worldwide. Its extent is estimated by imaging studies upon diagnosis, but they are useful only when there is a massive extraocular dissemination. Histopathological examination of the enucleated eye is used to evaluate microscopic invasion of the choroid, the optic nerve and the sclera, and postoperative therapy is decided upon this information. Since extraocular dissemination is uncommon in developed countries, there are few studies that provided evidence to support adjuvant therapy. When there is overt extraocular disease, children should be treated with neoadjuvant therapy followed by delayed enucleation and adjuvant therapy. The treatment of extraocular retinoblastoma and the controversies in its management are the subject of this review.