Primary metastatic Ewing's family tumors: Results of the Italian Sarcoma Group and Scandinavian Sarcoma Group ISG/SSG IV Study including myeloablative chemotherapy and total-lung irradiation

R. Luksch, A. Tienghi, K. Sundby Hall, F. Fagioli, P. Picci, E. Barbieri, L. Gandola, M. Eriksson, P. Ruggieri, P. Daolio, P. Lindholm, A. Prete, G. Bisogno, A. Tamburini, G. Grignani, M. E. Abate, M. Podda, S. Smeland, S. Ferrari

Research output: Contribution to journalArticlepeer-review

Abstract

Background: The Italian Sarcoma Group and the Scandinavian Sarcoma Group designed a joint study to improve the prognosis for patients with Ewing's family tumors and synchronous metastatic disease limited to the lungs, or the pleura, or a single bone. Patients and methods: The study was opened in 1999 and closed to the enrollment in 2008. The program consisted of intensive five-drug combination chemotherapy, surgery and/or radiotherapy as local treatment, and consolidation treatment with high-dose busulfan/melphalan plus autologous stem cell rescue and total-lung irradiation. Results: During the study period, 102 consecutive patients were enrolled. The median follow-up was 62 months (range 24-124). The 5-year event-free survival probability was 0.43 [standard deviation (SD) = 0.05] and the 5-year overall survival probability was 0.52 (SD = 0.052). Unfavorable prognostic factors emerging on multivariate analysis were a poor histological/radiological response at the site of the primary tumor [relative risk (RR) = 3.4], and incomplete radiological remission of lung metastases after primary chemotherapy (RR = 2.6). One toxic death and one secondary leukemia were recorded. Conclusions: This intensive approach is feasible and long-term survival is achievable in ∼50% of patients. New treatment approaches are warranted for patients responding poorly to primary chemotherapy.

Original languageEnglish
Article numbermds117
Pages (from-to)2970-2976
Number of pages7
JournalAnnals of Oncology
Volume23
Issue number11
DOIs
Publication statusPublished - Nov 2012

Keywords

  • Ewing's sarcoma
  • High-dose chemotherapy
  • Lung metastases
  • Total-lung irradiation

ASJC Scopus subject areas

  • Oncology
  • Hematology

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