Atypical teratoid rhabdoid tumor medical therapy: Difference between revisions

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Latest revision as of 21:16, 16 May 2019

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.

Template:WH Template:WS

[SlavcChocholous2014-1] 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.

[chemoatrt1-2] 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

[GinnGajjar2012-3] 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.

[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.

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@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Atypical teratoid rhabdoid tumor}}
-{{CMG}}
+{{CMG}}{{AE}}{{SR}}
 ==Overview==
-The mainstay of therapy for atypical teratoid rhabdoid tumor is combined [[chemotherapy]] and [[radiotherapy]].
+The predominant [[therapy]] for atypical teratoid rhabdoid tumor is [[Resection|surgical resection]]. Adjunctive [[chemotherapy]] and [[Radiation therapy|radiation]] is required.
 ==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>
+<br>
+{{familytree/start |summary=Treatment of atypical teratoid rhabdoid tumor}}
+{{familytree |boxstyle=background: #DCDCDC;| | | | | | | | | | | | | A01| | | | | | | | | | | | | |A01=<div style="width: 10em; padding:0.2em;">'''Treatment of atypical teratoid rhabdoid tumor'''</div>}}
+{{familytree |boxstyle=background: #DCDCDC;| | | | | | | |,|-|-|-|v|-|^|-|v|-|-|-|.| | | | | | | | | | | | | | |}}
+{{familytree |boxstyle=background: #DCDCDC;| | | | | | | B01 | | B02 | | B03 | | B04 | | | | | | | | | | | | | |B01=<div style="width: 9em; padding:0.2em;">'''Surgery''' </div>|B02=<div style="width: 9em; padding:0.2em;">'''Radiation therapy'''</div>|B03=<div style="width: 9em; padding:0.2em;">'''Chemotherapy'''</div>|B04=<div style="width: 9em; padding:0.2em;">'''Stem cell transplantation'''</div>}}
+{{familytree/end}}
 ===Chemotherapy ===
-Approximately 50% of the AT/RTs will transiently respond, but [[Chemotherapy]] by itself is rarely curative.  There is no standard treatment for AT/RT.  Various chemotherapeutic agents have been used against AT/RTs which are also used against other CNS tumors including [[cisplatin]]um, [[carboplatinum]], [[cyclophosphamide]], [[vincristine]] and [[etoposide]].  Some [[chemotherapy regimens|Chemotherapy protocols]] are listed below:
+*[[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 [[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>
-* CCG clinical trial CCG-9921 was activated in 1993 and published its results in 2005.  The proposed treatments did not have different outcomes and were not an improvement on prior treatments.<ref name="Geyer">{{cite journal |author=Geyer JR, Sposto R, Jennings M, ''et al'' |title=Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from the Children's Cancer Group |journal=J. Clin. Oncol. |volume=23 |issue=30 |pages=7621–31 |year=2005 |pmid=16234523 |doi=10.1200/JCO.2005.09.095 |url=http://jco.ascopubs.org/cgi/content/abstract/23/30/7621}}</ref> Geyer published a review of chemotherapy on 299 infants with CNS tumors that evaluated response rate, event-free survival (EFS), and toxicity of two chemotherapeutic regimens for treatment of children younger than 36 months with malignant brain tumors.  Patients were randomly assigned to one of two regimens of induction chemotherapy (vincristine, cisplatin, cyclophosphamide, and etoposide v vincristine, carboplatin, ifosfamide, and etoposide). Intensified induction chemotherapy resulted in a high response rate of malignant brain tumors in infants. Survival was comparable to that of previous studies, and most patients who survived did not receive radiation therapy.<ref name="Geyer" />
+*Approximately 50% of the atypical teratoid rhabdoid tumors will transiently respond, but [[chemotherapy]] by itself is rarely [[Cure|curative]].
-*[[Sarcoma]] protocols. There has been at least one report in the literature of malignant rhabdoid tumors of the CNS being treated in as a high-grade intracranial [[sarcoma]].  These three cases were treated with surgery, chemotherapy, radiotherapy and triple [[intrathecal]] chemotherapy similar to the Intergroup Rhadbdomyoscarcoma Study III guidelines.<ref>{{cite web |url=http://www.cancer.gov/cancertopics/pdq/treatment/childrhabdomyosarcoma/healthprofessional/ |title=Childhood Rhabdomyosarcoma Treatment|publisher= National Cancer Institute |accessdate=2007-07-09 }}</ref>
-* [[Intrathecal]] protocols. One of the difficulties with brain and spinal tumors is that the [[blood brain barrier]] needs to be crossed so that the drug can get to the tumor.  One mechanism to deliver the drug is through a device called an [[Ommaya reservoir]].  This is a device which shares some characteristics with a shunt in which a tube a surgically placed in the fluid surrounding the brain and a bulb shaped reservoir attached to the tubing is placed under the skin of the scalp.  When the child is to receive [[intrathecal]] [[chemotherapy]], the drug is administered into this bulb reservoir.   At other times intrathecal chemotherapeutic agents are delivered through a [[lumbar puncture]] (spinal tap). A current Pediatric Brain Tumor Consortium [[Chemotherapy regimens|Protocol]] uses intrathecal [[mafosfamide]], a pre-activated [[cyclophosphamide]]derivative, in addition to other modalities to try to effect this tumor.<ref>{{cite journal |author=Poussaint TY, Phillips PC, Vajapeyam S, ''et al'' |title=The Neuroimaging Center of the Pediatric Brain Tumor Consortium-collaborative neuroimaging in pediatric brain tumor research: a work in progress |journal=AJNR.  American journal of neuroradiology |volume=28 |issue=4 |pages=603–7 |year=2007 |pmid=17416804 |doi=}}</ref>
-* High dose chemotherapy with stem cell rescue. This therapy uses chemotherapy at doses high enough to completely suppress the [[bone marrow]].  Prior to instituting this therapy, the child has a [[Central venous catheter|central line]] placed and [[stem cell|stem cells]] are gathered.  After therapy these cells are given back to the child to [[Hematopoietic stem cell transplantation|regrow the bone marrow]].  Stem cell rescue or [[autologous]] [[bone marrow transplantation]], was initially thought to be of benefit to a wide group of patients, but has declined over the [[History of cancer chemotherapy|history of chemotherapy]] protocols.  A general description of stem cell rescue is available.<ref>{{cite web |url=http://www.cancerbackup.org.uk/Treatments/Stemcellbonemarrowtransplants/Generalinformation |title=Stem cell and bone marrow transplants|publisher= Cancerbackup |accessdate=2007-07-09 }}</ref>    In addition, there are some reports that it is effective with select cancers and this includes AT/RT.<ref>{{cite web |url=http://content.nejm.org/cgi/content/abstract/348/19/1875 |publisher=NEJM|title= High-Dose Chemotherapy with Hematopoietic Stem-Cell Rescue for Multiple Myeloma |accessdate=2007-07-09}}</ref>
 ===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.<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>
+*[[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 tumor|brain tumors]] has been to use [[chemotherapy]] to defer [[radiation therapy]], until a child is older than three years.
-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.
+*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 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 beam intersects at the tumor location; the normal brain tissue receives less radiation and hopefully is at less impact on cognitive function.
+*[[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]].
-[[Proton therapy|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.<ref>[http://www.massgeneral.org/cancer/about/providers/radiation/proton/principles.asp Principles of Proton Beam Therapy]</ref><ref>[http://neurosurgery.mgh.harvard.edu/ProtonBeam/  Proton Beam RadioTheraphy at Mass. General]</ref><ref>[http://www.ihf-publications.org/html/images/pdfs_ref2/107-117.pdf  Proton Beam Therapy Article]</ref><ref>[http://www.nature.com/bjc/journal/v93/n8/abs/6602754a.html  Proton Beam Therapy - BJC Abstract]</ref><ref>[http://www.llu.edu/proton/patient/index.html Loma Linda Medical Center Proton Treatment Center - Overview]</ref><ref>[http://www.llu.edu/proton/patient/sites.html#BRAIN Loma Linda overview of Childhood Brain Tumors]</ref>
 ==References==
 {{reflist|2}}
-{{WH}}
-{{WS}}
 [[Category:Types of cancer]]
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 [[Category:Pediatric cancers]]
 [[Category:Disease]]
+[[Category:Oncology]]
+{{WH}}
+{{WS}}