Abstract | PURPOSE: To retrospectively review an institutional experience in managing atypical teratoid/ rhabdoid tumors (AT/RT) of the Central Nervous System with high-dose chemotherapy in infants and children less than 4 years old. MATERIALS/METHODS: Eight AT/RT patients were identified during the study period 2003 to 2008. Tumor location was supratentorial in 3 cases, infratentorial in 3 cases, and multifocal in 2 patients. Five patients presented with leptomeningeal dissemination. Two of these patients did not receive any active therapy. After surgery, the 6 remaining patients received induction therapy followed by sequential high-dose chemotherapy with autologous stem cell rescue. Two patients receive focal irradiation. RESULTS: At a median follow-up of 52 months, 4 patients are alive without evidence of tumor. Three of these patients, including 2 with metastatic disease were not irradiated. However, all surviving patients exhibit neuro- cognitive impairment, either at baseline assessment or upon follow-up. CONCLUSIONS: This experience confirms that a subset of young AT/RT patients may achieve long-term survival with intensive and high-dose chemotherapy. The role of radiotherapy is still unclear. However, evidence of neuro- cognitive impairment even in the absence of radiotherapy in this young population suggests that systematic introduction of radiotherapy in current protocols should be carefully assessed.
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Authors | Tal Finkelstein-Shechter, Adam Gassas, Donald Mabbott, Annie Huang, Ute Bartels, Uri Tabori, Laura Janzen, Janzen Laura, Cynthia Hawkins, Michael Taylor, Eric Bouffet |
Journal | Journal of pediatric hematology/oncology
(J Pediatr Hematol Oncol)
Vol. 32
Issue 5
Pg. e182-6
(Jul 2010)
ISSN: 1536-3678 [Electronic] United States |
PMID | 20495479
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Vincristine
- Etoposide
- Cyclophosphamide
- Cisplatin
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Topics |
- Antineoplastic Combined Chemotherapy Protocols
(therapeutic use)
- Brain Neoplasms
(drug therapy, pathology, radiotherapy)
- Child, Preschool
- Cisplatin
(administration & dosage)
- Combined Modality Therapy
- Cranial Irradiation
- Cyclophosphamide
(administration & dosage)
- Etoposide
(administration & dosage)
- Female
- Humans
- Infant
- Male
- Neoplasm Metastasis
- Radiotherapy Dosage
- Retrospective Studies
- Rhabdoid Tumor
(drug therapy, pathology, radiotherapy)
- Survival Rate
- Teratoma
(drug therapy, pathology, radiotherapy)
- Treatment Outcome
- Vincristine
(administration & dosage)
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