Abstract

Controversy exists about the treatment outcomes of the Ewing sarcoma family of tumors among low-incidence populations. We evaluated whether Korean Ewing sarcoma family of tumors patients have poorer outcomes than Euro-American patients. We retrospectively analyzed the clinicopathologic characteristics and outcomes of patients with localized Ewing sarcoma family of tumors treated at Korea Cancer Center Hospital between 1986 and 2008. Seventy-six patients (48 male, 28 female) of median age 20 years (range: 1-69 years) were evaluated. Tumors were located in central-axial parts of the body in 33 cases (43.4%) and extremity in 43 cases (56.6%). Pelvis and femur were the most frequently involved sites. Histologic response to preoperative chemotherapy was analyzed in 48 cases and there were 32 (66.7%) good responders and 16 (33.3%) poor responders. For a median follow-up of 37.9 months (range: 0.9-260.6 months), 5-year overall survival and event-free survival rates were 58.9 ± 6.1 and 52.6 ± 6.1%, respectively. A poor histologic response to preoperative chemotherapy (P= 0.01) and a tumor location in a central-axial body region (P= 0.008) were found to be related to a poorer event-free survival. Survival of our Ewing sarcoma family of tumors patients was not inferior to those reported for Euro-American cases. Collaborative studies are necessary for further improvements of outcome and we believe that our data provide a basis for future studies targeting Ewing sarcoma family of tumors.

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