Atypical teratoid rhabdoid tumor medical therapy

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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

The predominant therapy for atypical teratoid rhabdoid tumor is surgical resection. Adjunctive chemotherapy and radiation is required.

Medical Therapy

The mainstay of therapy for atypical teratoid rhabdoid tumor includes:[1]

 
 
 
 
 
 
 
 
 
 
 
 
Treatment of atypical teratoid rhabdoid tumor
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Surgery
 
Radiation therapy
 
Chemotherapy
 
Stem cell transplantation
 
 
 
 
 
 
 
 
 
 
 
 
 


Chemotherapy

  • Chemotherapy is given intrathecally for the direct action of the drug on the central nervous system.[2]
  • Th various chemotherapeutic drugs used in the treatment of atypical teratoid rhabdoid tumor include cisplatin, cyclophosphamide, vincristine, and etoposide.[3]
  • Approximately 50% of the atypical teratoid rhabdoid tumors will transiently respond, but chemotherapy by itself is rarely curative.

Radiation Therapy

The traditional practice for childhood brain tumors has been to use chemotherapy to defer radiation therapy until a child is older than three years. This strategy is based upon observations that children under three have significant long term complications as a result of brain irradiation. However, the long term outcomes of AT/RT are so poor that protocols call for upfront radiation therapy, often in spite of young age.[4]

The dose and volume of radiation had not been standardized, however, radiation does appear to improve survival. The use of radiation has been limited in children younger than three because of the risk of severe neurocognitive deficits. There are protocols using conformal, local radiation in the young child to try to cure this tumor.

External beam (conformal) radiation uses several fields that beam intersects at the tumor location; the normal brain tissue receives less radiation and hopefully is at less impact on cognitive function.

Proton beam radiation was only offered at Massachusetts General Hospital in Boston and at Loma Linda, California as of 2002. Since 2003 three or four more proton therapy centers have opened in the United States.[5][6][7][8][9][10]

References

  1. 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.
  2. Symptoms and diagnosis of atypical teratoid rhabdoid tumor. Dana-Farber and Boston Children hospital cancer and blood disorder center 2015. http://www.danafarberbostonchildrens.org/conditions/brain-tumor/atypical-teratoid-rhabdoid-tumor.aspx. Accessed on Dcember 21, 2015
  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. Squire SE, Chan MD, Marcus KJ (2007). "Atypical teratoid/rhabdoid tumor: the controversy behind radiation therapy". J. Neurooncol. 81 (1): 97–111. doi:10.1007/s11060-006-9196-z. PMID 16855864.
  5. Principles of Proton Beam Therapy
  6. Proton Beam RadioTheraphy at Mass. General
  7. Proton Beam Therapy Article
  8. Proton Beam Therapy - BJC Abstract
  9. Loma Linda Medical Center Proton Treatment Center - Overview
  10. Loma Linda overview of Childhood Brain Tumors

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