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

Jump to navigation Jump to search
VisualWikitext
No edit summary
 
(26 intermediate revisions by 5 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Atypical teratoid rhabdoid tumor}}
{{Atypical teratoid rhabdoid tumor}}
{{CMG}}
{{CMG}}{{AE}}{{SR}}
 
==Overview==
==Overview==
==Natural History, Complications and Prognosis==
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%.
===Prognosis===
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.


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


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).   
===Natural History===
*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>


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.
===Complications===
*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>
:*[[Obstructive hydrocephalus]]
:*[[Leptomeningeal]] dissemination
:*[[hemorrhage|Intratumoral hemorrhage]]
:*Recurrence


The longest term survivals reported in the literature are:
===Prognosis===
* (a) Hilden and associates reported a child who was still free from disease at 46 months from diagnosis.<ref> {{cite journal
*[[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>
|author=Hilden JM, Meerbaum S, Burger P, ''et al''
*[[Patient|Patients]] usually succumb to atypical teratoid rhabdoid tumor between 6 months and 1 year from [[diagnosis]].
|title=Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry
*Compared to [[medulloblastoma]], atypical teratoid rhabdoid tumor has a significantly worse [[prognosis]].
|journal=J. Clin. Oncol.
*Older age at [[diagnosis]] is a favorable [[Prognosis|prognostic]] factor for atypical teratoid rhabdoid tumor.
|volume=22
*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>
|issue=14
:*[[Germline mutation]]
|pages=2877–84
:*Age < 2 years
|year=2004
:*[[Metastasis|Metastases]] at [[diagnosis]]
|pmid=15254056
:*Subtotal [[resection]]
|doi=10.1200/JCO.2004.07.073
:*Large [[Tumor|tumors]]
|url=  http://jco.ascopubs.org/cgi/content/full/22/14/2877?ck=nck
:*Recurrence
| 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}}
{{WH}}
 
{{WS}}
[[Category:Oncology]]
[[Category:Types of cancer]]
[[Category:Types of cancer]]
[[Category:Rare cancers]]
[[Category:Rare cancers]]
Line 134: Line 42:
[[Category:Pediatric cancers]]
[[Category:Pediatric cancers]]
[[Category:Disease]]
[[Category:Disease]]
[[Category:Oncology]]
{{WH}}
{{WS}}

Latest revision as of 18:39, 16 May 2019

Atypical teratoid rhabdoid tumor Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Atypical Teratoid Rhabdoid Tumor from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Atypical teratoid rhabdoid tumor natural history, complications and prognosis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Atypical teratoid rhabdoid tumor natural history, complications and prognosis

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Atypical teratoid rhabdoid tumor natural history, complications and prognosis

CDC on Atypical teratoid rhabdoid tumor natural history, complications and prognosis

Atypical teratoid rhabdoid tumor natural history, complications and prognosis in the news

Blogs on Atypical teratoid rhabdoid tumor natural history, complications and prognosis

Directions to Hospitals Treating Atypical teratoid rhabdoid tumor

Risk calculators and risk factors for Atypical teratoid rhabdoid tumor natural history, complications and prognosis

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

Template:WH Template:WS

Retrieved from "https://www.wikidoc.org/index.php?title=Atypical_teratoid_rhabdoid_tumor_natural_history,_complications_and_prognosis&oldid=1568573"