2020–2021 BCSC Basic and Clinical Science Course™
4 Ophthalmic Pathology and Intraocular Tumors
Part II: Intraocular Tumors: Clinical Aspects
Chapter 19: Retinoblastoma
Retinoblastoma is the most common primary intraocular malignant tumor of childhood and is the second most common primary intraocular malignant tumor in all age groups (after uveal melanoma). The frequency of retinoblastoma in the United States and Europe ranges from 1 in 14,000 to 1 in 20,000 live births, with an estimated 250–300 new cases occurring each year. Both sexes and all races are affected equally, and the tumor occurs bilaterally in 30%–40% of patients. Approximately 90% of retinoblastoma cases are diagnosed in patients younger than 3 years. The mean age or age range at diagnosis is dependent on family history and disease laterality:
patients with known family history of retinoblastoma: 4–8 months
patients with bilateral disease: 12 months
patients with unilateral disease: 24 months
The annual worldwide incidence of retinoblastoma is 8000–9000 cases, and the disease rate varies among countries by approximately 50-fold, mainly because of variances in birthrates. Disease registries indicate that the highest incidences of retinoblastoma occur in India, China, and countries in Africa.
Abramson DH, Beaverson K, Sangani P, et al. Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival. Pediatrics. 2003;112(6 Pt. 1):1248–1255.
Orjuela M. Epidemiology. In: Rodriguez-Galindo C, Wilson MW, eds. Retinoblastoma. Springer; 2010:11–23. Pediatric Oncology.
Wong JR, Tucker MA, Kleinerman RA, Devesa SS. Retinoblastoma incidence patterns in the US Surveillance, Epidemiology, and End Results program. JAMA Ophthalmol. 2014;132(4):478–483.
Excerpted from BCSC 2020-2021 series: Section 4 - Ophthalmic Pathology and Intraocular Tumors. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.