Retinoblastoma: An International Perspective

Abstract

Retinoblastoma presents unique challenges to treating physicians in developing countries. The burden of caring for 80% of the world’s retinoblastoma cases falls to individuals and national health-care systems with limited resources where caring for children with extraocular disease is relatively common. Extraocular dissemination is the leading cause of mortality in this setting, so in many less developed countries there are more children dying of retinoblastoma than those surviving it. Because of the increased prevalence of extraocular dissemination, retinoblastoma specialists from developing countries have taken the lead in creating an International Staging system for extraocular retinoblastoma and in standardizing pathology reports in order to uniform risk assignment under evidence-based guidelines. Eye conservative treatments are a major challenge in less developed countries. However, successful programs using intravenous and more recently intra-arterial chemotherapy have been launched in middle-income countries. The major problem worldwide for conservative therapy is lack of acceptance of the enucleation of affected eyes failing treatment, especially when the fellow eye has been removed.

Prospective international studies for the treatment of high-risk disease were done in middle-income countries with encouraging results. Understanding the cause(s) of nonheritable or environmental retinoblastoma will likely take place in countries outside of North America and Europe. Initiatives that lead to early diagnosis, improved compliance for treatment, and improved quality of medical care of retinoblastoma patients in developing countries will likely result in improved outcomes. International collaborative efforts would further improve outcomes.