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 mainstay of therapy for atypical teratoid rhabdoid tumor is combined chemotherapy and radiotherapy.
Medical Therapy
The mainstay of therapy for atypical teratoid rhabdoid tumor includes:[1]
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 cisplatinum, carboplatinum, cyclophosphamide, vincristine and etoposide. Some Chemotherapy protocols are listed below:
- 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.[2] 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.[2]
- 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.[3]
- 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 Protocol uses intrathecal mafosfamide, a pre-activated cyclophosphamidederivative, in addition to other modalities to try to effect this tumor.[4]
- 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 line placed and stem cells are gathered. After therapy these cells are given back to the child to 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 chemotherapy protocols. A general description of stem cell rescue is available.[5] In addition, there are some reports that it is effective with select cancers and this includes AT/RT.[6]
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.[7]
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.[8][9][10][11][12][13]
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.
- ↑ 2.0 2.1 Geyer JR, Sposto R, Jennings M; et al. (2005). "Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from the Children's Cancer Group". J. Clin. Oncol. 23 (30): 7621–31. doi:10.1200/JCO.2005.09.095. PMID 16234523.
- ↑ "Childhood Rhabdomyosarcoma Treatment". National Cancer Institute. Retrieved 2007-07-09.
- ↑ Poussaint TY, Phillips PC, Vajapeyam S; et al. (2007). "The Neuroimaging Center of the Pediatric Brain Tumor Consortium-collaborative neuroimaging in pediatric brain tumor research: a work in progress". AJNR. American journal of neuroradiology. 28 (4): 603–7. PMID 17416804.
- ↑ "Stem cell and bone marrow transplants". Cancerbackup. Retrieved 2007-07-09.
- ↑ "High-Dose Chemotherapy with Hematopoietic Stem-Cell Rescue for Multiple Myeloma". NEJM. Retrieved 2007-07-09.
- ↑ 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.
- ↑ Principles of Proton Beam Therapy
- ↑ Proton Beam RadioTheraphy at Mass. General
- ↑ Proton Beam Therapy Article
- ↑ Proton Beam Therapy - BJC Abstract
- ↑ Loma Linda Medical Center Proton Treatment Center - Overview
- ↑ Loma Linda overview of Childhood Brain Tumors