[Epithelioid sarcoma: clinical behavior, prognostic factors and survival]

To relate the clinical characteristics and evaluations of patients with epithelioid sarcomas.

Careful analysis of 25 epithelioid sarcoma cases registered in Instituto Nacional do Cancer between june 1987 and july 2005.

Mean age at diagnosis was 33 years old, ranged from 10 to 70. The primary site of presentation was the upper extremity in twelve patients (48%). The size of the tumor was given in 19 cases, with the mean size of 5 cm, while they ranged from 1.5 to 15 cm. Surgery was made in 17 patients, with eleven amputation. Tumors margins were free in fifteen patients, positive in three and in seven were not studied. Six received any type of chemotherapy and 14 received treatment with radiotherapy with mean dose of 46,5 Gy. Local recurrence occurred in thirteen cases (52%). Nodal spread was diagnosed in nine (36%). Pulmonary metastases were diagnosed in seven patients (28%). Six patients underwent cancer treatment in its entirety at the National Institute of Cancer. At present twelve patients are alive without disease, two have disease and eleven patients have died.

Epithelioid sarcoma is a rare subset of soft tissue sarcoma with high rate of local recurrence, regional node and distant metastases. Occurs predominantly in young patients, mainly on the superior member extremities. Surgical treatment of epithelioid sarcoma consists of early wide local resection to negative microscopic margins. These patients require carefully follow-up to evaluate local recurrence, nodal metastases, and pulmonary metastases.