Non-metastatic Ewing's sarcoma family of tumors of bone in adolescents and adults: prognostic factors and clinical outcome-single institution results

• Published in: Tumori Vol. 100; no. 4; pp. 452 - 458
• Main Authors: Oksüz, Didem Colpan, Tural, Deniz, Dincbas, Fazilet Öner, Dervisoglu, Sergülen, Turna, Hande, Hiz, Murat, Kantarci, Fatih, Ceylaner, Beyhan, Koca, Sedat, Mandel, Nil Molinas
• Format: Journal Article
• Language: English
• Published: United States 01-07-2014
• Subjects: Adolescent; Adult; Age Factors; Bone Neoplasms - pathology; Bone Neoplasms - radiotherapy; Bone Neoplasms - surgery; Bone Neoplasms - therapy; Disease-Free Survival; Female; Follow-Up Studies; Humans; Kaplan-Meier Estimate; Male; Middle Aged; Neoadjuvant Therapy - methods; Predictive Value of Tests; Prognosis; Radiotherapy, Adjuvant; Risk Assessment; Risk Factors; Sarcoma, Ewing - pathology; Sarcoma, Ewing - radiotherapy; Sarcoma, Ewing - surgery; Sarcoma, Ewing - therapy; Treatment Outcome; Young Adult
• ISSN: 0300-8916; 2038-2529
• DOI: 10.1177/1636.17910

There is limited data regarding outcomes of Ewing'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's sarcoma family of tumors.
From 1992-2008, 90 adolescents and adults with Ewing'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.
The median age was 21 years (range, 13-50). Most patients (74%) were >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 >17 years of age, tumor size >8 cm in diameter, an axial location, positive surgical margins, and poor histopathological response (<90% necrosis). Age, tumor site and tumor size on event-free and overall survival remained significant on multivariate analysis.
We identified age, tumor size, and tumor site as independent prognostic factors, in accord with the Western literature. These patients require novel treatment modalities.