Epidemiology

The incidence of retinoblastoma is 1 in 12,000 to 30,000 live births.[16][17]​​ In the US, the annual retinoblastoma incidence rates (Surveillance, Epidemiology, and End Results [SEER] Program; 1973 to 2012) were approximately 12 cases per 1 million children 4 years or younger.[18][19]

In a prospective cluster-based analysis of 4064 retinoblastoma patients from 149 countries, the Global Retinoblastoma Study Group reported a median age at diagnosis (between Jan 1, 2017, and Dec 31, 2017) of 13.6 months in high-income countries, and 30 months in low-income countries.[20]​ In a subsequent cross-sectional analysis by the Global Retinoblastoma Study Group, approximately 50% of patients from low-income countries had extraocular retinoblastoma at diagnosis (median age at diagnosis of 30.5 months).[9]

The clinical stage of presentation, and laterality, of retinoblastoma may influence survival. Previous studies have demonstrated that unilateral retinoblastoma is characterized by late presentation and worse ocular outcome.[21][22]​​ Unilateral retinoblastoma is the most common form of the disease in low-, middle-, and high-income countries.[9]​[20]​​

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