Nonmetastatic Ewing family tumors: high-dose chemotherapy with stem cell rescue in poor responder patients. Results of the Italian Sarcoma Group/Scandinavian Sarcoma Group III protocol

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Background: High-dose chemotherapy (HDT) was added to conventional chemotherapy in Ewing sarcoma family tumor (EFT) patients, poor responders (PRs) to induction chemotherapy in order to improve their survival. Patients and methods: Patients aged <= 40 years with nonmetastatic Ewing sarcoma (ES) received vincristine (V), doxorubicin (A), cyclofosfamide (C), actinomycin (Ac), ifosfamide (I) and etoposide (E) (VACAc-IE regimen) as induction chemotherapy. As maintenance treatment, good responders (GR) received nine cycles of VACAc-IE regimen. PRs received three cycles of VAC-IE, mobilizing cycle with CE and HDT with Busulfan and Melphalan with stem cell support. Results: Three hundred patients [median age 15 years (3-40 years)] entered the study. One patient refused local treatment, 242 (81%) underwent surgery [with radiotherapy (RT) in 80] and 57 (19%) RT alone. No toxic deaths were recorded. Overall GR were 146 (49%). Twenty-eight PR did not receive HDT. At a median follow-up of 64 months (21-116 months), 5-year overall and event-free survival (EFS) were 75% and 69%, respectively. Five-year EFS was 75% for GR, 72% for PR treated with HDT and 33% for PR who did not receive HDT. Conclusions: High-dose therapy added to the VACA-IE regimen in PR patients is feasible and effective. Selected groups of patients with ES can benefit from HDT.


  • S. Ferrari
  • K. Sundby Hall
  • R. Luksch
  • A. Tienghi
  • Thomas Wiebe
  • F. Fagioli
  • Thor Alvegård
  • A. Brach del Prever
  • A. Tamburini
  • M. Alberghini
  • L. Gandola
  • M. Mercuri
  • R. Capanna
  • S. Mapelli
  • A. Prete
  • M. Carli
  • P. Picci
  • E. Barbieri
  • G. Bacci
  • S. Smeland
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Cancer and Oncology


  • chemotherapy-induced necrosis, Ewing sarcoma, high-dose chemotherapy
Original languageEnglish
Pages (from-to)1221-1227
JournalAnnals of Oncology
Issue number5
Publication statusPublished - 2011
Publication categoryResearch