Epithelioid sarcoma: the University of Washington experience

Background Epithelioid sarcoma is a rare sarcoma with a high local recurrence rate that frequently metastasizes to lymph nodes. We reviewed our experience with adjuvant therapy in patients with this disease. Methods Between 1990 and 2003, we treated 11 patients with epithelioid sarcoma. Patient, tumor, and treatment characteristics were analyzed, and effect of treatment on survival was evaluated by the Kaplan-Meier method. Results Nine men and 2 women were treated. Tumors presented on the trunk, the upper extremities, and the lower extremities. Five patients developed nodal disease. All patients underwent surgery for the primary tumor, and 7 patients had nodal evaluation. Ten patients underwent adjuvant chemotherapy, and 9 underwent radiotherapy. Recurrence developed in 9 patients. Five-year disease-free and overall survival rates were 46% and 65%, respectively. Chemotherapy and radiation therapy did not impact disease-free survival. Conclusions Although surgery remains the primary treatment modality, multi-institutional trials are needed to develop more effective adjuvant therapy for patients with epithelioid sarcoma.