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

Jump to navigation Jump to search
VisualWikitext
Line 44: Line 44:
**Metastases at diagnosis
**Metastases at diagnosis
**Subtotal resection
**Subtotal resection
 
**Large tumors
 
*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==

Revision as of 17:38, 16 December 2015

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

Natural History

Complications

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.

One case of metastatic disease to the abdomen via ventriculoperitoneal shunt has been reported with AT/RT . Metastatic dissemination via this mechanism has been reported with other brain tumors including germinomas, medulloblastomas, astrocytomas, glioblastomas, ependymomas and endodermal sinus tumors. Guler and Sugita separately reported cases of lung metastasis without a shunt.[1][2]

Prognosis

  • Prognosis is generally poor, and the 5-year overall survival rate of patients with atypical teratoid rhabdoid tumor is 29 ± 9%.[3]
  • The 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
  • Older age at diagnosis is a favorable prognostifc factor for atypical teratoid rhabdoid tumor.
  • Poor prognostic factors for atypical teratoid rhabdoid tumor include:[4]
    • Germline mutation
    • Age < 2 years
    • Metastases at diagnosis
    • Subtotal resection
    • Large tumors
  • Recurrence

References

  1. ↑ Güler E, Varan A, Söylemezoglu F; et al. (2001). "Extraneural metastasis in a child with atypical teratoid rhabdoid tumor of the central nervous system" (PDF). J. Neurooncol. 54 (1): 53–6. PMID 11763423.
  2. ↑ Sugita Y, Takahashi Y, Hayashi I, Morimatsu M, Okamoto K, Shigemori M (1999). "Pineal malignant rhabdoid tumor with chondroid formation in an adult". Pathol. Int. 49 (12): 1114–8. PMID 10632935.
  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=1195148"