A multimodal strategy based on surgery, radiotherapy, ICE regimen and high dose chemotherapy in atypical teratoid/rhabdoid tumours: A single institution experience

Paola Fidani, Maria Antonietta De Ioris, Annalisa Serra, Luigi De Sio, Ilaria Ilari, Raffaele Cozza, Renata Boldrini, Giuseppe Maria Milano, Maria Luisa Garrè, Alberto Donfrancesco

Research output: Contribution to journalArticle

Abstract

Purpose: Atypical Teratoid/Rhabdoid Tumour is a rare and aggressive childhood tumour. The outcome of a series treated with the same multimodal strategy was reported. Patients: The patients were treated with surgery, 2 courses of ifosfamide/carboplatin/etoposide(ICE), 2 courses of cyclophosphamide/ etoposide/carboplatino/thiotepa (CECAT) or 2 other ICE courses, high dose chemotherapy (HDC) and radiotherapy. Results: Eight patients underwent primary surgery achieving a complete removal in 3. Progressive disease (PD) occurred in 2/8 patients during ICE courses and in 3/4 during CECAT courses. After 4 courses 5 patients presented a PD. HDC was performed in 3 patients followed by local radiotherapy. The Kaplan Meier OS and EFS probability at 5 years are, respectively, 50% (CI 11-80%) and 33% (CI 6-66%). Conclusion: A strategy based on surgery, including a second surgical look, and on radiotherapy appears the best option. ICE regimen and HDC correlate with good prognosis in some patients but this approach needs further evaluation.

Original languageEnglish
Pages (from-to)177-183
Number of pages7
JournalJournal of Neuro-Oncology
Volume92
Issue number2
DOIs
Publication statusPublished - 2009

Keywords

  • Atypical teratoid/rhabdoid tumour
  • High dose chemotherapy
  • ICE regimen
  • INI1
  • Survival

ASJC Scopus subject areas

  • Clinical Neurology
  • Cancer Research
  • Oncology
  • Neurology

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