
Health Dictionary
Reliable, easy-to-read patient information on common medical conditions β written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Retinoblastoma epidemiology and demographics
Introduction
Overview
Overview
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.
Epidemiology and Demographics
Epidemiology and Demographics
Incidence
- 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.
Prevalence
- There is no data on the prevalence of retinoblastoma.
Case-fatality rate/Mortality rate
- 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]
Age
- 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.
Gender
Race
Region
- 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:
| Country | Incidence |
|---|---|
| Mali | 4.25 |
| Uganda | 2.4 |
| Zimbabwe | 2.33 |
| Hawaii | 2.25 |
| India | 1.96 |
| Vietnam | 1.89 |
| Singapore | 1.88 |
| New Zealand | 1.78 – 1.86 |
| Spain | 1.78 |
| Philippines | 1.74 |
| Colombia | 1.71 |
| Ecuador | 1.66 |
| Nigeria | 1.61 |
| Costa Rica | 1.57 |
| Peru | 1.55 |
References
References
- β Fernandes, Arthur Gustavo; Pollock, Benjamin D.; Rabito, Felicia A. (2018). “Retinoblastoma in the United States: A 40-Year Incidence and Survival Analysis”. Journal of Pediatric Ophthalmology & Strabismus. 55 (3): 182β188. doi:10.3928/01913913-20171116-03. ISSNΒ 0191-3913.
- β Dimaras, Helen; Kimani, Kahaki; Dimba, Elizabeth AO; Gronsdahl, Peggy; White, Abby; Chan, Helen SL; Gallie, Brenda L (2012). “Retinoblastoma”. The Lancet. 379 (9824): 1436β1446. doi:10.1016/S0140-6736(11)61137-9. ISSNΒ 0140-6736.
- β Kim, J W; Kathpalia, V; Dunkel, I J; Wong, R K; Riedel, E; Abramson, D H (2008). “Orbital recurrence of retinoblastoma following enucleation”. British Journal of Ophthalmology. 93 (4): 463β467. doi:10.1136/bjo.2008.138453. ISSNΒ 0007-1161.
- β ChΓ©vez-Barrios, Patricia; Eagle, Ralph C.; Marback, Eduardo F. (2015). “Histopathologic Features and Prognostic Factors”: 167β183. doi:10.1007/978-3-662-43451-2_16.
- β 5.0 5.1 5.2 Abramson DH, Frank CM, Susman M, Whalen MP, Dunkel IJ, Boyd NW (1998). “Presenting signs of retinoblastoma”. J Pediatr. 132 (3 Pt 1): 505β8. PMIDΒ 9544909.
- β Broaddus E, Topham A, Singh AD (2009). “Incidence of retinoblastoma in the USA: 1975-2004”. Br J Ophthalmol. 93 (1): 21β3. doi:10.1136/bjo.2008.138750. PMIDΒ 18621794.
- β Binder PS (1974). “Unusual manifestations of retinoblastoma”. Am J Ophthalmol. 77 (5): 674β9. PMIDΒ 4132770.
- β Zakka KA, Yee RD, Foos RY (1983). “Retinoblastoma in a 12-year-old girl”. Ann Ophthalmol. 15 (1): 88β91. PMIDΒ 6830100.
- β Kaliki S, Shields CL, Gupta A, Mishra DK, Das C, Say EA, Shields JA (December 2015). “NEWLY DIAGNOSED ACTIVE RETINOBLASTOMA IN ADULTS”. Retina (Philadelphia, Pa.). 35 (12): 2483β8. doi:10.1097/IAE.0000000000000612. PMIDΒ 26035399.
- β Singh, Arun (2007). Clinical ophthalmic oncology. Edinburgh: Elsevier Saunders. ISBNΒ 978-1-4160-3167-3.
β
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]
