Abstract
Atypical teratoid rhabdoid tumors (ATRT) are highly malignant tumors of the central nervous system with a peak incidence in children less than 3 years of age. Despite multimodal therapy including surgery, radiation and chemotherapy, the prognosis remains dismal. No specific treatment guidelines are defined for ATRTs but a gross total resection and radiation therapy (RT) appear to improve overall outcome. In children less than 3 years of age, the prognosis is dismal due in part to the reluctance to utilize RT given its severe neurological sequelae. To avoid RT in this age group, intensification of chemotherapy has been tried and has shown to improve outcome. Myeloablative chemotherapy followed by autologous stem cell re-infusion has been used as a modality to intensify therapy but there are no reports of use of tandem myeloablative regimens and autologous stem cell re-infusions for treatment of ATRT. We herein report the case of a 4-month-old boy with ATRT with partial resection of his tumor who achieved complete remission using tandem high-dose therapy followed by autologous peripheral blood stem cell re-infusions despite having biopsy proven disease at the time of starting the tandem regimens. This was achieved without the use of RT as a treatment modality.
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Jemal A, Clegg LX, Ward E et al (2004) Annual report to the nation on the status of cancer, 1975–2001, with a special feature regarding survival. Cancer 101:3–27
Squire SE, Chan MD, Marcus KJ (2007) Atypical teratoid/rhabdoid tumor: the controversy behind radiation therapy. J Neurooncol 81(1):97–111
Reddy AT (2005) Atypical teratoid/rhabdoid tumors of the central nervous system. J Neurooncol 75(3):309–313
Judkins AR, Mauger J, Bielgel JA et al (2004) Immunohistochemical analysis of hSNF5/INI1 in pediatric CNS neoplasms. Am J Surg Pathol 28(5):644–650
Chi SN, Gardener SL, Levy AS et al (2004) Feasibility and response to induction chemotherapy intensified with high dose methotrexate for young children with newly diagnosed high-risk disseminated medulloblastoma. J Clin Oncol 22(24):4881–4887
Zimmerman MA, Goumnerova LC, Proctor M et al (2005) Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor. J Neurooncol 72(1):77–84
Olson TA, Bayar E, Kosnik E et al (1995) Successful treatment of disseminated central nervous system malignant rhabdoid tumor. J Pediatr Hematol Oncol 17(1):71–75
Tekautz TM, Fuller CE, Blaney S et al (2005) Atypical teratoid/rhabdoid tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator based chemotherapy. J Clin Oncol 23(7):1491–1499
Hilden JM, Watterson J, Longee DC et al (1998) Central nervous system atypical teratoid/rhabdoid tumor: response to intensive therapy and review of the literature. J Neuro Oncol 40(3):265–275
Beschorner R, Mittlebronn M, Koerbel A et al (2006) Atypical teratoid-rhabdoid tumor spreading along the trigeminal nerve. Pediatr Neurosurg 42(4):258–263
Fujita M, Sato M, Nakamura M et al (2005) Multicentric atypical teratoid/rhabdoid tumors occurring in the eye and fourth ventricle of an infant. J Neurosurg 102(3 suppl):299–302
Ronghe MD, Moss TH, Lowis SP (2004) Treatment of CNS malignant rhabdoid tumors. Pediatr Blood Cancer 42(3):254–260
Hilden JM, Meerbaum S, Burger P et al (2004) Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry. J Clin Oncol 22(14):2877–2884
Katzenstein HM, Kletzel M, Reynolds M et al (2003) Metastatic malignant rhabdoid tumor of the liver treated with tandem high-dose therapy and autologous peripheral blood stem cell rescue. Med Pediatr Oncol 40(3):199–201
Perez-Martinez A, Lassaletta A, Gonzalez-Vincent M et al (2005) High-dose chemotherapy with autologous stem cell rescue for children with high risk and recurrent medulloblastoma and supratentorial primitive neuroectodermal tumors. J Neurooncol 71(1):33–38
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Gidwani, P., Levy, A., Goodrich, J. et al. Successful outcome with tandem myeloablative chemotherapy and autologous peripheral blood stem cell transplants in a patient with atypical teratoid/rhabdoid tumor of the central nervous system. J Neurooncol 88, 211–215 (2008). https://doi.org/10.1007/s11060-008-9553-1
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DOI: https://doi.org/10.1007/s11060-008-9553-1