Atypical teratoid rhabdoid tumor medical therapy: Difference between revisions
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==Overview== | ==Overview== | ||
The predominant therapy for atypical teratoid rhabdoid tumor is surgical resection. Adjunctive chemotherapy and radiation is required. | The predominant [[therapy]] for atypical teratoid rhabdoid tumor is [[Resection|surgical resection]]. Adjunctive [[chemotherapy]] and [[Radiation therapy|radiation]] is required. | ||
==Medical Therapy== | ==Medical Therapy== | ||
The mainstay of therapy for atypical teratoid rhabdoid tumor includes:<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref> | The mainstay of [[therapy]] for atypical teratoid rhabdoid tumor includes:<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref> | ||
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{{familytree/start |summary=Treatment of atypical teratoid rhabdoid tumor}} | {{familytree/start |summary=Treatment of atypical teratoid rhabdoid tumor}} | ||
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===Chemotherapy === | ===Chemotherapy === | ||
*[[Chemotherapy]] is given intrathecally for the direct action of the drug on the central nervous system.<ref name=chemoatrt1>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</ref> | *[[Chemotherapy]] is given [[Intrathecal|intrathecally]] for the direct action of the [[:Category:Drugs|drug]] on the [[central nervous system]].<ref name="chemoatrt1">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</ref> | ||
*Various chemotherapeutic drugs used in the treatment of atypical teratoid rhabdoid tumor include [[cisplatin]], [[cyclophosphamide]], [[vincristine]], and [[etoposide]].<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> | *Various [[Chemotherapy|chemotherapeutic]] [[:Category:Drugs|drugs]] used in the treatment of atypical teratoid rhabdoid tumor include [[cisplatin]], [[cyclophosphamide]], [[vincristine]], and [[etoposide]].<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> | ||
*Approximately 50% of the atypical teratoid rhabdoid tumors will transiently respond, but [[chemotherapy]] by itself is rarely curative. | *Approximately 50% of the atypical teratoid rhabdoid tumors will transiently respond, but [[chemotherapy]] by itself is rarely [[Cure|curative]]. | ||
===Radiation Therapy=== | ===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.<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> | *[[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.<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 traditional practice for childhood brain tumors has been to use chemotherapy to defer [[radiation therapy]], until a child is older than three years. | *The traditional practice for childhood [[Brain tumor|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.<ref>{{cite journal |author=Squire SE, Chan MD, Marcus KJ |title=Atypical teratoid/rhabdoid tumor: the controversy behind radiation therapy |journal=J. Neurooncol. |volume=81 |issue=1 |pages=97–111 |year=2007 |pmid=16855864 |doi=10.1007/s11060-006-9196-z}}</ref> | *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.<ref>{{cite journal |author=Squire SE, Chan MD, Marcus KJ |title=Atypical teratoid/rhabdoid tumor: the controversy behind radiation therapy |journal=J. Neurooncol. |volume=81 |issue=1 |pages=97–111 |year=2007 |pmid=16855864 |doi=10.1007/s11060-006-9196-z}}</ref> | ||
*The dose and volume of radiation have not been standardized, however, radiation appears to improve survival. | *The [[dose]] and volume of [[Radiation therapy|radiation]] have not been standardized, however, [[Radiation therapy|radiation]] appears to improve survival. | ||
*[[External beam radiotherapy|External beam]] (conformal) radiation uses several fields that intersects the beam at the tumor location; the normal brain tissue receives less radiation and | *[[External beam radiotherapy|External beam]] (conformal) [[Radiation therapy|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 [[Cognition|cognitive function]]. | ||
==References== | ==References== | ||
Latest revision as of 21:16, 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
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.