Abstract
9038 Background: The best treatment for Extra-Osseous Ewing Sarcoma (EOE) is still matter of debate Methods: Between 1989 and 1999, according to countries, patients (pts) with EOE were treated either in SIOP MMT 89 study (32 pts) or SFOP osseous Ewing's tumor (OE) study (31 pts). Briefly, MMT strategy included chemotherapy (IVA: Ifosfamide-Vincristine-Actinomycine, 6 to 9 courses), surgical resection in pts with incomplete response to chemotherapy (CT) and local irradiation in case of post-operative residue. OE protocol included CT (Cyclophosphamide-Doxorubicin, CPM-DXR, 5 courses), surgical resection; then, treatment was stratified on histological response: good responders (< 10% viable cells) received alternating courses of CPM-DXR and Vincristine-Actino during 6 months. Four poor responders were given intensified chemotherapy (Ifosfamide-VP16) before high-dose chemotherapy (Bu-Mel) followed by local irradiation for 3 of them. Results: Characteristics of pts treated in both protocols were similar in terms of age and sites. Five-year OS and EFS of the pts treated with OE protocol were 83 and 75% respectively, which was significantly better than OS and EFS of the pts treated with MMT strategy (59 and 44%) (p=0.04 and 0.008 respectively). Although local treatment was more intensive in OE strategy (96% resections vs 75%, 71% irradiations vs 50%), the difference in the outcome is explained by a higher rate of metastatic relapses in pts treated with MMT protocol (Metastasis-free survival of 82 vs 54%). Univariate analysis for overall survival showed that age < 10 years, and OE protocol were favourable predictive factors. Univariate analysis for disease-free survival showed that size < 5 cm and OE protocol were favourable predictive factors. However, in multivariate analysis, only type of protocol remained significant for overall and disease-free survivals. Conclusions: Our study suggests that patients with EOE should be treated with bony targeted regimen, probably because they contain anthracyclins No significant financial relationships to disclose.
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