Atypical teratoid or rhabdoid tumors: improved outcome with high-dose chemotherapy.

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.
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.
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.
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.
AuthorsTal Finkelstein-Shechter, Adam Gassas, Donald Mabbott, Annie Huang, Ute Bartels, Uri Tabori, Laura Janzen, Janzen Laura, Cynthia Hawkins, Michael Taylor, Eric Bouffet
JournalJournal of pediatric hematology/oncology (J Pediatr Hematol Oncol) Vol. 32 Issue 5 Pg. e182-6 (Jul 2010) ISSN: 1536-3678 [Electronic] United States
PMID20495479 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Vincristine
  • Etoposide
  • Cyclophosphamide
  • Cisplatin
  • 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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