Abstract

Epithelioid sarcoma (ES) has a tendency to locally recur, spread proximally, and metastasize, in particular to lymphnodes and lungs. The aim of this report is to study the role of surgery and the extent of margins required for optimal management of patients with localized epithelioid sarcoma of the extremities. We retrospectively evaluated 77 patients affected by ES of the extremities treated at two different Institutions. Twenty-two patients had metastasis at diagnosis. Estimated survival was 65.5% at 5 years and 50.9% at 10 years, with a better prognosis in patients with localized disease at diagnosis (P < 0.001). Among patients with localized disease, a significantly better survival was found in patients with primary tumors in which radical surgical margins were achieved (P = 0.043). Among 47 patients presenting with primary tumors, local recurrence-free rate was 72.9% at 5 years, and 61.9% at 10 years, with a better local control achieved in patients with radical margins were achieved (P = 0.026). We believe that the best approach to improve both local control and survival is to aim for radical margins in patients with primary tumors. Therefore, the best chance for cure is if the first treatment is the right treatment, which we believe to be radical margins.

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