摘要

Aims and background. There is limited data regarding outcomes of Ewing%26apos;s sarcoma family of tumors in adolescents and adults compared with the same tumors in childhood. The aim of the study was to analyze prognostic factors and treatment results in a cohort of adolescents and adults with non-metastatic skeletal Ewing%26apos;s sarcoma family of tumors. %26lt;br%26gt;Methods and study design. From 1992-2008, 90 adolescents and adults with Ewing%26apos;s sarcoma family of tumors of the bone were referred to our institution. Sixty-five (72%) non-metastatic patients with analyzable data and treated in our institution were retrospectively evaluated. All patients were treated with alternated chemotherapy regimens administered every 3 weeks. The local treatment modality was selected according to tumor and patient characteristics. %26lt;br%26gt;Results. The median age was 21 years (range, 13-50). Most patients (74%) were %26gt;17 years of age. Forty-six percent of the tumors were located in the extremities. Local therapy was surgery in 45 patients and radiotherapy alone in 19 patients. Twenty-one patients received preoperative and 13 patients postoperative radiotherapy. Median follow-up was 43 months (range, 7-167). The 5-year event-free and overall survival rates for all patients were 44% and 49%, respectively. On univariate survival analysis, event-free and overall survival were worse for patients %26gt;17 years of age, tumor size %26gt;8 cm in diameter, an axial location, positive surgical margins, and poor histopathological response (%26lt;90% necrosis). Age, tumor site and tumor size on event-free and overall survival remained significant on multivariate analysis. %26lt;br%26gt;Conclusions. We identified age, tumor size, and tumor site as independent prognostic factors, in accord with the Western literature. These patients require novel treatment modalities.

  • 出版日期2014-8