Atypical teratoid rhabdoid tumor natural history, complications and prognosis: Difference between revisions

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==Overview==
==Overview==
If left untreated, [[Patient|patients]] with atypical teratoid rhabdoid tumor may progress to develop [[obstructive hydrocephalus]], [[leptomeningeal]] dissemination, and ultimately death. Common [[Complication (medicine)|complications]] of atypical teratoid rhabdoid tumor include [[obstructive hydrocephalus]], [[leptomeningeal]] dissemination, and recurrence. [[Prognosis]] is generally poor, and the 5-year overall [[survival rate]] of [[Patient|patients]] with atypical teratoid rhabdoid tumor is 29 ± 9%.


==Natural History==
== Natural History, Complications, and Prognosis ==


==Complications==
===Natural History===
Metastatic spread is noted in approximately one-third of the AT/RT cases at the time of diagnosis and tumors can occur anywhere throughout the CNS.  The ASCO study of the 188 documented AT/RT cases prior to 2004 found 30% of the cases had metastasis at diagnosis. Metastatic spread to the [[meninges]] (leptomenigeal spread sometimes referred to as sugar coating) is common both initially and with relapse. Average survival times decline with the presence of metastasis.  Primary CNS tumors metastasize only within the CNS.
*If left untreated, [[Patient|patients]] with atypical teratoid rhabdoid tumor may progress to develop [[obstructive hydrocephalus]], [[leptomeningeal]] dissemination, and ultimately death.<ref name="pmid22135607">{{cite journal| author=Chan KH, Mohammed Haspani MS, Tan YC, Kassim F| title=A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl. | journal=Malays J Med Sci | year= 2011 | volume= 18 | issue= 3 | pages= 82-6 | pmid=22135607 | doi= | pmc=PMC3216225 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22135607 }} </ref>


One case of metastatic disease to the abdomen via ventriculoperitoneal [[shunt (medical)|shunt]] has been reported with AT/RT .  Metastatic dissemination via this mechanism has been reported with other brain tumors including [[germinoma]]s, [[medulloblastoma]]s, [[astrocytoma]]s, [[Glioma|glioblastomas]], [[Ependymoma|ependymomas]] and [[endodermal sinus tumor]]s.  Guler and Sugita separately reported cases of lung metastasis without a shunt.<ref>{{cite journal
===Complications===
|author=Güler E, Varan A, Söylemezoglu F, ''et al''
*Common [[Complication (medicine)|complications]] of atypical teratoid rhabdoid tumor include:<ref name="pmid22135607">{{cite journal| author=Chan KH, Mohammed Haspani MS, Tan YC, Kassim F| title=A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl. | journal=Malays J Med Sci | year= 2011 | volume= 18 | issue= 3 | pages= 82-6 | pmid=22135607 | doi= | pmc=PMC3216225 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22135607  }} </ref><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>
|title=Extraneural metastasis in a child with atypical teratoid rhabdoid tumor of the central nervous system
:*[[Obstructive hydrocephalus]]
|journal=J. Neurooncol.
:*[[Leptomeningeal]] dissemination
|volume=54
:*[[hemorrhage|Intratumoral hemorrhage]]
|issue=1
:*Recurrence
|pages=53–6
|year=2001
|pmid=11763423
|doi=
|url=http://www.kluweronline.com/art.pdf?issn=0167-594X&volume=54&page=53
}}</ref><ref>{{cite journal
|author=Sugita Y, Takahashi Y, Hayashi I, Morimatsu M, Okamoto K, Shigemori M
|title=Pineal malignant rhabdoid tumor with chondroid formation in an adult
|journal=Pathol. Int.
|volume=49
|issue=12
|pages=1114–8
|year=1999
|pmid=10632935
|doi=
|url=http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=1320-5463&date=1999&volume=49&issue=12&spage=1114
}}</ref>


==Prognosis==
===Prognosis===
*Prognosis is generally poor, and the 5-year overall survival rate of patients with atypical teratoid rhabdoid tumor is 29 ± 9%.<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>
*[[Prognosis]] is generally poor, and the 5-year overall [[survival rate]] of [[Patient|patients]] with atypical teratoid rhabdoid tumor is 29 ± 9%.<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>
*The patients usually succumb to atypical teratoid rhabdoid tumor between 6 months and 1 year from diagnosis.
*[[Patient|Patients]] usually succumb to atypical teratoid rhabdoid tumor between 6 months and 1 year from [[diagnosis]].
*Compared to [[medulloblastoma]], atypical teratoid rhabdoid tumor has a significantly worse prognosis
*Compared to [[medulloblastoma]], atypical teratoid rhabdoid tumor has a significantly worse [[prognosis]].
*Older age at diagnosis is a favorable prognostifc factor for atypical teratoid rhabdoid tumor.
*Older age at [[diagnosis]] is a favorable [[Prognosis|prognostic]] factor for atypical teratoid rhabdoid tumor.
*Poor prognostic factors for atypical teratoid rhabdoid tumor include:<ref name=progatrt1>Prognosis of atypical teratoid rhabdoid tumor. National Cancer Institute 2015. http://www.cancer.gov/types/brain/hp/child-cns-atrt-treatment-pdq. Accessed on December 14, 2015</ref>
*Poor [[Prognosis|prognostic]] factors for atypical teratoid rhabdoid tumor include:<ref name="progatrt1">Prognosis of atypical teratoid rhabdoid tumor. National Cancer Institute 2015. http://www.cancer.gov/types/brain/hp/child-cns-atrt-treatment-pdq. Accessed on December 14, 2015</ref>
**Germline mutation
:*[[Germline mutation]]
**Age < 2 years
:*Age < 2 years
**Metastases at diagnosis
:*[[Metastasis|Metastases]] at [[diagnosis]]
**Subtotal resection
:*Subtotal [[resection]]
**Large tumors
:*Large [[Tumor|tumors]]
**Recurrence
:*Recurrence


==References==
==References==
{{reflist|2}}
{{reflist|2}}
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Latest revision as of 18:39, 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

If left untreated, patients with atypical teratoid rhabdoid tumor may progress to develop obstructive hydrocephalus, leptomeningeal dissemination, and ultimately death. Common complications of atypical teratoid rhabdoid tumor include obstructive hydrocephalus, leptomeningeal dissemination, and recurrence. Prognosis is generally poor, and the 5-year overall survival rate of patients with atypical teratoid rhabdoid tumor is 29 ± 9%.

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

References

  1. ↑ 1.0 1.1 Chan KH, Mohammed Haspani MS, Tan YC, Kassim F (2011). "A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl". Malays J Med Sci. 18 (3): 82–6. PMC 3216225. PMID 22135607.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ Prognosis of atypical teratoid rhabdoid tumor. National Cancer Institute 2015. http://www.cancer.gov/types/brain/hp/child-cns-atrt-treatment-pdq. Accessed on December 14, 2015

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