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]].
*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>
*Compared to [[medulloblastoma]], atypical teratoid rhabdoid tumor has a significantly worse [[prognosis]].
**Germline mutation
*Older age at [[diagnosis]] is a favorable [[Prognosis|prognostic]] factor for atypical teratoid rhabdoid tumor.
**Age < 2 years
*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>
**Metastases at diagnosis
:*[[Germline mutation]]
**Subtotal resection
:*Age < 2 years
 
:*[[Metastasis|Metastases]] at [[diagnosis]]
Most patients with AT/RT are less than three years old, the overall prognosis for AT/RT is very poor. Compared to medulloblastoma, AT/RT has a significantly worse prognosis.  AT/RT occurs in young children (often younger than three years) who are difficult to evaluate, it is resistant to many current therapies, and its recurrence is fast.
:*Subtotal [[resection]]
 
:*Large [[Tumor|tumors]]
The prognosis for AT/RT is very poor, although there are some indications that an IRSIII-based therapy can produce long-term survival (60 to 72 months).  Two-year survival is less than 20%, average survival postoperatively is 11 months, and doctors recommend palliative care, especially with younger children because of the poor outcomes.
:*Recurrence
 
Patients with metastasis (disseminated tumor), larger tumors, tumors that could not be fully removed, tumor reoccurrence, and were younger than 36 months had the worse outcomes (i.e., shorter survival times). 
 
A retrospective survey from 36 AT/RT St. Jude Children's Hospital patients from 1984 to 2004 found a less than 10% survival rate in children under three, but a 70% survival rate in older children.<ref>{{cite web |url=http://www.stjude.org/disease-summaries/0,2557,449_2160_7267,00.html |title=Atypical Teratoid / Rhabdoid Tumor (ATRT) |accessdate=2007-07-10 |publisher=St Jude's Hospital}}</ref> A retrospective register at the Cleveland Children's hospital on 42 AT/RT patients found median survival time is 16.25 months and a survival rate around 33%. One-quarter of these cases did not show the mutation in the [[SWI/SNF|INI1/hSNF5]] gene.
 
The longest term survivals reported in the literature are:
* (a) Hilden and associates reported a child who was still free from disease at 46 months from diagnosis.<ref> {{cite journal
|author=Hilden JM, Meerbaum S, Burger P, ''et al''
|title=Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry
|journal=J. Clin. Oncol.
|volume=22
|issue=14
|pages=2877–84
|year=2004
|pmid=15254056
|doi=10.1200/JCO.2004.07.073
|url=  http://jco.ascopubs.org/cgi/content/full/22/14/2877?ck=nck
| accessdate = 2007-05-23}}</ref>
* (b) Olson and associates reported a child who was disease free at five years from diagnosis based on the IRS III protocol. <ref>  {{cite journal
  | author =  Olson TA, Bayar E, Kosnic E
  | title =  Successful treatment of disseminated central nervous system malignant rhabdoid tumors
  | journal = J Pediatr Hematol Oncol
  | volume =  17
  | pages =  71–75
  | pmid = 7743242
  | year =  1995  }}</ref>
* (c) In 2003 Hirth reported a case who had been disease free for over six years.<ref>{{cite journal
| author = Hirth A, Pedersen P-H, Wester K, et al
| year = 2003
| title = Cerebral Atypical Teratoid/Rhabdoid Tumor of Infancy: Long-Term Survival after Multimodal Treatment, also Including Triple Intrathecal Chemotherapy and Gamma Knife Radiosurgery--Case Report (Abstract)
| journal =  Pediatric Hematology and Oncology 2003
| volume = 20
| issue = 4
| pages = 327–332
|DOI = 10.1080/713842315
| url = http://www.informaworld.com/smpp/content~content=a713842315~db=all
}} </ref>
* (d)  Zimmerman in 2005 reported 50-to-72 month survival rates on four patients using an IRS III-based protocol.  Two of these LT survivors had been treated after an AT/RT reoccurrence.<ref>{{cite journal
|author=Zimmerman MA, Goumnerova LC, Proctor M, ''et al''
|title=Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor
|journal=J. Neurooncol.
|volume=72
|issue=1
|pages=77–84
|year=2005
|pmid=15803379
|doi=10.1007/s11060-004-3115-y
|url=  http://www.ingentaconnect.com/content/klu/neon/2005/00000072/00000001/00003115
| accessdate = 2007-05-20}}</ref>
* (e) A NYU study (Gardner 2004) has 4 of 12 longer term AT/RT survivors; the oldest was alive at 46 months after diagnosis.<ref>{{cite journal
  | author =  Gardner S, Diez B, Green A, et al
  | title =  THER 27. INTENSIVE INDUCTION CHEMOTHERAPY FOLLOWED BY HIGH-DOSE CHEMOTHERAPY WITH AUTOLOGOUS STEM CELL RESCUE (ASCR) IN YOUNG CHILDREN NEWLY DIAGNOSED WITH CENTRAL NERVOUS SYSTEM (CNS) ATYPICAL TERATOID RHABDOID TUMORS (ATT/RT)—THE “HEAD START” REGIMENS
  | journal = Abstracts from the Eleventh International Symposium on Pediatric Neuro-Oncology
  | year =  2004
  | date=June 13–16
  | url =  http://neuro-oncology.dukejournals.org/cgi/reprint/6/4/401.pdf
  | accessdate = 2007-06-03}}</ref>
* (f) Aurélie Fabre, 2004, reported a 16-year survivor of a soft-tissue rhabdoid tumor.<ref>{{cite journal
  | author =  Fabre A, Eyden B, Ali HH
  | title =  Soft-Tissue Extrarenal Rhabdoid Tumor with a Unique Long-Term Survival
  | journal =  Ultrastructural Pathology
  | volume = 28
  | issue = 1
  | date= January 2004
  | year =  2004
  | pages =  49–52
  | DOI = 10.1080/01913120490275259
  | url =  http://www.informaworld.com/smpp/content~content=a714860453~db=all
  | accessdate = 2007-05-28}}</ref>
 
Cancer treatments in long-term survivors who are children usually cause a series of negative effects on physical well being, fertility, cognition, and [[Learning problems in childhood cancer|learning]].<ref>
{{cite journal
  | author =  Fouladi M, Gilger E, Kocak M, et al
  | title =  Intellectual and Functional Outcome of Children 3 Years Old or Younger Who Have CNS Malignancies
  | journal =  Journal of Clinical Oncology
  | volume = 23
  | number =  28
  | date= [[October 1]], [[2005]]
  | pages = 7152–60
  | DOI =  10.1200/JCO.2005.01.214 
  | url = http://jco.ascopubs.org/cgi/content/full/23/28/7152}}</ref><ref>
{{cite journal
  | author =  Monteleone P, Meadows AT
  | title =  Late Effects of Childhood Cancer and Treatment
  | journal =  eMedicine from WebMD
  | date= [[June 6]], [[2006]]
  | year = 2006
  | url = http://www.emedicine.com/ped/topic2591.htm}}</ref><ref>
{{cite journal
  | author =  Foreman NK, Faestel PM, Pearson J, et al
  | title =  Health Status in 52 Long-term Survivors of Pediatric Brain Tumors
  | journal =  Journal of Neuro-Oncology
  | date= January 1999
  | year = 1999
  | volume = 41
  | number = 1
  | pages = 47–52
  | DOI  = 10.1023/A:1006145724500
  | url = http://www.springerlink.com/content/n651371xh3q8g42t/}}
</ref><ref>
{{cite journal 
  |author = Meyers EA, Kieran MW
  |title = Brief Report Psychological adjustment of surgery-only pediatric neuro-oncology patients: a retrospective analysis 
  |journal = Psycho-Oncology
  |publisher = John Wiley & Sons, Ltd.
  |year =  2002
  |volume =  11
  |number =  1
  |pages =  74–79
  |DOI = 10.1002/pon.553
  |url  =  http://www3.interscience.wiley.com/cgi-bin/abstract/89016824/ABSTRACT?CRETRY=1&SRETRY=0}}
</ref>


==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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