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
Clinical study - patient study

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.

Keywords

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

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Copyright information

© Springer Science+Business Media, LLC. 2008

Authors and Affiliations

  • Paola Fidani
    • 1
  • Maria Antonietta De Ioris
    • 1
  • Annalisa Serra
    • 1
  • Luigi De Sio
    • 1
  • Ilaria Ilari
    • 1
  • Raffaele Cozza
    • 1
  • Renata Boldrini
    • 2
  • Giuseppe Maria Milano
    • 1
  • Maria Luisa Garrè
    • 3
  • Alberto Donfrancesco
    • 1
  1. 1.Division of Pediatric Oncology UnitOspedale Pediatrico Bambino GesùRomeItaly
  2. 2.Department of PathologyOspedale Pediatrico Bambino GesùRomeItaly
  3. 3.Division of Pediatric Haematology and OncologyOspedale G.GasliniGenoaItaly

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