Retinoblastoma epidemiology and demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2] Simrat Sarai, M.D. [3]

The incidence of retinoblastoma in the United States has been reported to be 1.2 cases per 100,000 children aged 4 years or younger. The median age at diagnosis of retinoblastoma is 18 months. The average age at diagnosis of retinoblastoma for children with unilateral disease and bilateral disease is 24 months and 12 months respectively. Retinoblastoma affects males and females equally. There is no racial predilection to the development of retinoblastoma.

• The incidence of retinoblastoma in the United States has been reported to be 1.2 cases per 100,000 children aged 4 years or younger.^[1]
• The tumor incidence has been reported 0.049 cases per 100,000 child aged 5 to 9.

• There is no data on the prevalence of retinoblastoma.

• The mortality rate of retinoblastoma differs according to the stage of the disease as well as the geographic region.^[2]
• In extraocular form of this disorder is reported to be greater than 50%.^[3]
• However, tumors involving the optic disc superficially, are associated with 10% mortality rate.^[4]

• The median age at the time of diagnosis is 18 months.^[5]
• The average age at diagnosis of retinoblastoma for children with unilateral disease and bilateral disease is 24 months and 12 months respectively.^[6]
• Cases of newly diagnosed retinoblastoma have been reported in children as old as 18 years and even in adults.^[7][8]
• In adults, retinoblastoma tends to present between 20 to 50 years of age.^[9]
• Trilateral retinoblastoma is a well-recognized syndrome that occurs in 5% to 15% of patients with heritable retinoblastoma and is defined by the development of an intracranial midline neuroblastic tumor, which typically develops between the ages of 20 and 36 months.

• Retinoblastoma affects males and females equally.^[5]

• There is no racial predilection to the development of retinoblastoma.^[5]

• Epidemiological data indicates that retinoblastoma has a higher incidence in some geographic areas.^[10]
• The table below provides the highest worldwide incidence rate of retinoblastoma in children aged 0 – 4:

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