Epithelioid sarcoma of the extremities: a dismal long-term outcome

Epithelioid sarcoma is a rare histologic subtype of sarcoma. The clinical behavior and prognostic factors influencing survival in this disease are examined. A review of clinicopathologic features of patients with epithelioid sarcoma prospectively followed between September 1981 and April 2001 at the Cancer Institute Hospital was performed. Eight patients (4 men and 4 women) constituted the subjects of this study, with a mean age of 41 years. Tumors presented in the lower extremity in 62.5% of patients and in the upper extremity in 37.5%. All patients were followed for at least 10 years from the time of diagnosis or until death. The follow-up ranged from 17 to 228 months, with a mean of 78 months. At least one local recurrence was seen in 50% of patients. During the course of the disease, metastases to regional lymph nodes developed in 50% of patients and metastases to the lungs in 62.5%. The median survival was 31 months, with a 25% 5-year and 10-year survival rate. Pulmonary metastases were correlated with decreased survival. A delay in diagnosis of epithelioid sarcoma is common. Epithelioid sarcoma differs from other sarcoma subtypes in its propensity for nodal spread and local recurrence. Chemotherapy and radiotherapy have an insignificant effect on the course of epithelioid sarcoma. Careful follow-up, evaluating local recurrence, nodal spread, and pulmonary metastases, is warranted. The long-term outcome of epithelioid sarcoma is dismal.