Italian cooperative study for the treatment of children and young adults with localized Ewing sarcoma of bone - A preliminary report of 6 years of experience

BACKGROUND. In 1991, the Italian Association for Pediatric Hematology-Oncol ogy smd the National Council of Research (CNR) initiated an Italian Coopera tive Study (SE 91-CNR Protocol) with the main objective of improving the ov erall survival (SUR) and the event free survival (EFS) of children and youn g adults with localized Ewing sarcoma and primitive neuroectodermal tumors of bone compared with a previous study (IOR/Ew2 Protocol). METHODS. Between November 1991 and November 1997, 165 patients were enrolle d in this study, 160 of whom were evaluable. The patients were treated with a multimodal approach characterized by intensified chemotherapy, hyperfrac tionated and accelerated radiation therapy, and the addition of ifosfamide and etoposide to standard chemotherapy with vincristine, actinomycin-D, dox onibicin, and cyclophosphamide. RESULTS. After a median follow-up of 37 months, 126 of the 160 evaluable pa tients remained free of disease recurrence. Thirty-one patients developed a disease recurrence (20 with disseminated disease). CONCLUSIONS. The 3-year SUR and EFS rates found in the current study (83.6% and 17.8%, respectively) may be considered satisfactory. Only age at diagn osis less than or equal to 14 years and a good histologic response appeared to affect the outcome of patients with localized Ewing sarcoma positively. These results appear to demonstrate the efficacy of the addition of ifosfa mide in induction chemotherapy to four-drug standard combination chemothera py, as confirmed by the improved outcome in terms of 3-year EFS reported in the SE 91-CNR Protocol compared with the IOR/Ew2 Protocol (77.8% vs. 60.7% ). In addition, the: better outcome also could be explained by the change i n treatment strategy with a trend toward the use of more surgery than radia tion therapy compared with the authors' previous protocol. (C) 1999 America n Cancer Society.