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]
- 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
- Radiation is an effective component of therapy for atypical teratoid rhabdoid tumor, but is avoided in patients younger than 3 years of age due to the long term neurocognitive sequelae.[3]
- The traditional practice for childhood brain tumors has been to use chemotherapy to defer radiation therapy, until a child is older than three years.
- However, the long term outcomes of atypical teratoid rhabdoid tumor are so poor that protocols call for upfront radiation therapy, often in spite of young age.[4]
- The dose and volume of radiation have not been standardized, however, radiation appears to improve survival.
- External beam (conformal) radiation uses several fields that intersects the beam at the tumor location; the normal brain tissue receives less radiation and has less impact on the cognitive function.
References
- ↑ 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.
- ↑ Treatment and care options 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 December 21, 2015
- ↑ 3.0 3.1 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.
- ↑ 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.