Retinoblastoma epidemiology and demographics
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Incidence and Prevalence
Here are some highlights listed by the SEER (Surveillance, Epidemiology and End Results) about the incidence of retinoblastoma among children and adolescents.[1]
- Retinoblastoma accounted for approximately 11% of cancers developing in the first year of life,but for only 3% of the cancers developing among children younger than 15 years of age.
- In the US, approximately 300 children and adolescents younger than 20 years of age are diagnosed with retinoblastomas each year.
- The vast majority of cases of retinoblastoma occur among young children with almost two-thirds(63%) of retinoblastomas occuring before the age of two years and 95% occuring before the age of 5 years.
- The incidence of bilateral tumors was strongly age dependent with 42% of the retinoblastomas occuring in children less than one year of age being bilateral compared to 21% of those among children aged one year, and only 9% among older children.
- Rates of retinoblastoma were the same among males (3.7 per million) and females (4.0 per million) and also among whites (3.7 per million) and blacks (4.0 per million).
- There was no subsantial change in the retinoblastoma incidence during the 21 year period, 1975-95.
- It is estimated that 38.2% of patients with hereditary retinoblastoma will develop a secondary malignancy with an associated long-term mortality rate of 26%.
- Retinoblastoma presents with cumulative lifetime incidence rate of 1 case of retinoblastoma per 18000 to 30000 live births worldwide. A higher incidence is noted in developing countries, this has been implicated to lower socioeconomic status and the presence of human papilloma virus sequences in the retinoblastoma tissue.
- Almost 80% of children with retinoblastoma are diagnosed before 3 years of age and diagnosis in children above 6 years of age is extremely rare. In the UK, bilateral cases usually present within 14–16 months, while diagnosis of unilateral cases peaks between 24 and 30 months.