Atypical teratoid rhabdoid tumor epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Epidemiology and Demographics
Incidence
- The incidence of atypical teratoid rhabdoid tumor is approximately 0.3 per 100,000 individuals in the United States.[1]
Age
- Atypical teratoid rhabdoid tumor is a rare disease that tends to affect the children population.[2]
- The median age at diagnosis is approximately 2-3 years.
In the United States, three children per 1,000,000 or around 30 new AT/RT cases are diagnosed each year. AT/RT represents around 3% of pediatric cancers of the central nervous system (CNS).[3]
Around 17% of all pediatric cancers involve the CNS; it is the most common childhood solid tumor. The survival rate for CNS tumors is around 60%; with AT/RT it is around 10%. Pediatric brain cancer is the second leading cause of childhood death, just after leukemia. Recent trends suggest that the rate of overall CNS tumor diagnosis is increasing by about 2.7% per year. As diagnostic techniques using genetic markers improve and are used more often, the proportion of AT/RT diagnoses is expected to increase.
Since it is highly malignant, AT/RT has a high mortality rate. A survey of 36 AT/RT patients at St. Jude Children's Hospital from 1984 to 2003 showed the survival rate for children under three is less than 10%, whereas for older children, the survival rate is potentially over 70%.[4] Because most patients with AT/RT are less than three years old, the overall prognosis for AT/RT is very poor.
An estimated 3% of pediatric brain tumors are AT/RTs although this percentage may increase with better differentiation between PNET/medulloblastoma tumors and AT/RTs. As with other CNS tumors, slightly more males are affected than females (ratio 1.6:1). The ASCO study showed a 1.4:1 male to female ratio.
References
- ↑ Atypical teratoid rhabdoid tumor. Wikipedia 2015. https://en.wikipedia.org/wiki/Atypical_teratoid_rhabdoid_tumor. Accessed on December 10, 2015
- ↑ Epidemiology of atypical teratoid rhabdoid tumor. Dr Bruno Di Muzio and Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 10, 2015
- ↑ Measure D6: Types of Childhood Cancer – 2006 Tables D6a & D6b. U.S. Environmental Protection Agency. Retrieved on 2008-04-17.
- ↑ Tekautz TM, Fuller CE, Blaney S; et al. (2005). "Atypical teratoid/rhabdoid tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy". J. Clin. Oncol. 23 (7): 1491–9. doi:10.1200/JCO.2005.05.187. PMID 15735125. See Figure 1.