Extraskeletal Ewing's sarcoma: A report of 18 cases andliterature review

Abstract

BACKGROUND AND OBJECTIVE: Extraskeletal Ewing's sarcoma (EES) is a rare, rapidly growing, round-cell, malignant tumor that can develop in the soft tissues at any location. This study was to analyze the clinical features, diagnosis and treatment of EES. METHODS: Clinical data of 18 patients with EES, treated at between Cancer Center of Sun Yat-sen University between 1995 and 2007, were analyzed. RESULTS: Of the 18 patients, 13 were male and 8 were female, aged from 8 months to 60 years. Twelve (66.7%) patients were between 5-25 years of age. Eight (44.4%) patients had tumors originated from low extremities. Sixteen patients had masses at their first visit. Sixteen patients were treated by the combined modality therapy, and 2 patients were treated by the single modality therapy. The 1-, 3- and 5- year actuarial survival rates were 82.4%, 64.2% and 32.1%, respectively. The presence of metastatic disease at the time of diagnosis and the mode of treatment were prognostic factors. CONCLUSIONS: EES is common in adolescent. It often manifests as a localized mass. The combined modality therapy is recommended for this disease. The presence of metastatic disease at the time of diagnosis and the mode of treatment are prognostic factors.