Retinoblastoma: A Global Perspective

Retinoblastoma presents unique challenges to treating physicians in low- and middle-income settings. 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 resourced-limited settings, there are more children dying of retinoblastoma than those surviving it. Because of the increased prevalence of extraocular dissemination, retinoblastoma specialists from low- and middle-income 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 low- and middle-income settings. 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 the 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 diseases were done in middle-income countries with encouraging results. Understanding the cause(s) of non-heritable 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.