Global Burden of Retinoblastoma

Retinoblastoma (Rb) is the most common pediatric eye cancer. While the incidence of Rb is constant across the world, its distribution is skewed, with most cases arising in low- and middle-income countries from Asia and Africa. The Global Retinoblastoma Presentation and Outcome Studies (2017–2020) uncovered, for the first time, the worldwide disparities in Rb presentation, treatment, and outcomes. In high-income countries, treatment for children with Rb is practically curable, with management focused primarily on the globe and vision salvage. However, in lower-income countries, late presentation and extraocular disease dominate, with mortality reaching 57% at three-year follow-up. Several independent sub-analyses of the Global Rb Studies have supplemented the original research, providing further insights into the geographic and socioeconomic discrepancies of Rb worldwide. In this book chapter, we review the study designs and significant outcomes of these studies and subsequent analyses, providing a comprehensive overview of the burden of Rb globally.