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==Overview==
==Overview==
Atypical teratoid rhabdoid tumor comprises approximately 1 - 2% of all [[Pediatrics|pediatric]] [[Brain tumor|brain tumors]]; however, in [[Patient|patients]] less than 3 years of age, this [[tumor]] accounts for up to 20% of cases. The [[incidence]] of atypical teratoid rhabdoid tumor is approximately 0.3 per 100,000 individuals in the United States. Atypical teratoid rhabdoid tumor is a rare [[disease]] that tends to affect the children. The [[median]] age at [[diagnosis]] is approximately 24 - 30 months. [[Male|Males]] are more commonly affected with atypical teratoid rhabdoid tumor than [[Female|females]]. The [[male]] to [[female]] ratio is approximately 1.9 to 1.


==Epidemiology and Demographics==
==Epidemiology and Demographics==
===Incidence===
*The [[incidence]] of atypical teratoid rhabdoid tumor is approximately 0.3 per 100,000 individuals in the United States.<ref name="atrtinci1">Atypical teratoid rhabdoid tumor. Wikipedia 2015. https://en.wikipedia.org/wiki/Atypical_teratoid_rhabdoid_tumor. Accessed on December 10, 2015</ref>
===Prevalence===
===Prevalence===
*Atypical teratoid rhabdoid tumor comprises approximately 1–2% of all pediatric brain tumors; however, in patients less than 3 years of age, this tumor accounts for up to 20% of cases.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref>
*Atypical teratoid rhabdoid tumor comprises approximately 1 - 2% of all [[Pediatrics|pediatric]] [[Brain tumor|brain tumors]]; however, in [[Patient|patients]] less than 3 years of age, this [[tumor]] accounts for up to 20% of cases.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref>


===Incidence===
===Age===
*The incidence of atypical teratoid rhabdoid tumor is approximately 0.3 per 100,000 individuals in the United States.<ref name=atrtinci1>Atypical teratoid rhabdoid tumor. Wikipedia 2015. https://en.wikipedia.org/wiki/Atypical_teratoid_rhabdoid_tumor. Accessed on December 10, 2015</ref>
*Atypical teratoid rhabdoid tumor is a rare [[disease]] that tends to affect the children.<ref name="epidemioageatrt1">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</ref><ref name="epioageatrt1">Clinical presentation of atypical teratoid rhabdoid tumor. National Cancer Institute 2015. http://www.cancer.gov/types/brain/hp/child-cns-atrt-treatment-pdq#link/_113_toc. Accessed on December 16, 2015</ref>
*However, although rare, atypical teratoid rhabdoid tumor has also been reported in [[adult]] [[Patient|patients]].<ref name="UdakaShayan2013">{{cite journal|last1=Udaka|first1=Y. T.|last2=Shayan|first2=K.|last3=Chuang|first3=N. A.|last4=Crawford|first4=J. R.|title=Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child|journal=Case Reports in Oncological Medicine|volume=2013|year=2013|pages=1–4|issn=2090-6706|doi=10.1155/2013/815923}}</ref>
*The [[median]] age at [[diagnosis]] is approximately 24 - 30 months.<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref>


===Age===
=== Race ===
*Atypical teratoid rhabdoid tumor is a rare disease that tends to affect the children population.<ref name=epidemioageatrt1>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</ref>
* There is no racial predilection to atypical teratoid rhabdoid tumor.
*The median age at diagnosis is approximately 2-3 years.


===Gender===
===Gender===
*Males are more commonly affected with atypical teratoid rhabdoid tumor than females. The male to female ratio is approximately 1.9 to 1.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref>
*[[Male|Males]] are more commonly affected with atypical teratoid rhabdoid tumor than [[Female|females]]. The [[male]] to [[female]] ratio is approximately 1.9 to 1.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref>
 
 
 
 
 
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 [[cancer]]s of the central nervous system (CNS).<ref>[http://www.epa.gov/envirohealth/children/child_illness/d6-graph.htm Measure D6:  Types of Childhood Cancer] – 2006 Tables D6a & D6b.  U.S. Environmental Protection Agency.  Retrieved on [[2008-04-17]].</ref>
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 [[cancer|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%.<ref>{{cite journal |author=Tekautz TM, Fuller CE, Blaney S, ''et al'' |title=Atypical teratoid/rhabdoid tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy |journal=[[J. Clin. Oncol.]] |volume=23 |issue=7 |pages=1491–9 |year=2005 |pmid=15735125 |doi=10.1200/JCO.2005.05.187 |url=http://jco.ascopubs.org/cgi/content/full/23/7/1491}} See Figure 1.</ref>  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==
==References==
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[[Category:Pediatric cancers]]
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[[Category:Disease]]
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[[Category:Needs overview]]
[[Category:Oncology]]
 
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Latest revision as of 18:00, 16 May 2019

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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]

Overview

Atypical teratoid rhabdoid tumor comprises approximately 1 - 2% of all pediatric brain tumors; however, in patients less than 3 years of age, this tumor accounts for up to 20% of cases. The incidence of atypical teratoid rhabdoid tumor is approximately 0.3 per 100,000 individuals in the United States. Atypical teratoid rhabdoid tumor is a rare disease that tends to affect the children. The median age at diagnosis is approximately 24 - 30 months. Males are more commonly affected with atypical teratoid rhabdoid tumor than females. The male to female ratio is approximately 1.9 to 1.

Epidemiology and Demographics

Incidence

  • The incidence of atypical teratoid rhabdoid tumor is approximately 0.3 per 100,000 individuals in the United States.[1]

Prevalence

  • Atypical teratoid rhabdoid tumor comprises approximately 1 - 2% of all pediatric brain tumors; however, in patients less than 3 years of age, this tumor accounts for up to 20% of cases.[2]

Age

  • Atypical teratoid rhabdoid tumor is a rare disease that tends to affect the children.[3][4]
  • However, although rare, atypical teratoid rhabdoid tumor has also been reported in adult patients.[5]
  • The median age at diagnosis is approximately 24 - 30 months.[6]

Race

  • There is no racial predilection to atypical teratoid rhabdoid tumor.

Gender

  • Males are more commonly affected with atypical teratoid rhabdoid tumor than females. The male to female ratio is approximately 1.9 to 1.[2]

References

  1. Atypical teratoid rhabdoid tumor. Wikipedia 2015. https://en.wikipedia.org/wiki/Atypical_teratoid_rhabdoid_tumor. Accessed on December 10, 2015
  2. 2.0 2.1 Ginn, Kevin F.; Gajjar, Amar (2012). "Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions". Frontiers in Oncology. 2. doi:10.3389/fonc.2012.00114. ISSN 2234-943X.
  3. 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
  4. Clinical presentation of atypical teratoid rhabdoid tumor. National Cancer Institute 2015. http://www.cancer.gov/types/brain/hp/child-cns-atrt-treatment-pdq#link/_113_toc. Accessed on December 16, 2015
  5. Udaka, Y. T.; Shayan, K.; Chuang, N. A.; Crawford, J. R. (2013). "Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child". Case Reports in Oncological Medicine. 2013: 1–4. doi:10.1155/2013/815923. ISSN 2090-6706.
  6. Slavc, Irene; Chocholous, Monika; Leiss, Ulrike; Haberler, Christine; Peyrl, Andreas; Azizi, Amedeo A.; Dieckmann, Karin; Woehrer, Adelheid; Peters, Christina; Widhalm, Georg; Dorfer, Christian; Czech, Thomas (2014). "Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012". Cancer Medicine. 3 (1): 91–100. doi:10.1002/cam4.161. ISSN 2045-7634.

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