Diagnosis and treatment of epithelioid sarcoma

Abstract

To analyze the clinical features, diagnosis, treatment, and prognosis of epithelioid sarcoma (ES). The clinical data of 13 cases with epithelioid sarcoma in the Tianjin Medical University Cancer Institute and Hospital from March 1995 to December 2009 were collected and analyzed. There were 10 males and 3 females in the group, with an average age of 41.5 years (range: 13 to 68 years). Nine patients had classic ES and 4 had proximal-type ES. Surgery was the mainstay of treatment. After the operation, four patients received radiotherapy, five received chemotherapy, and one received chemoradiotherapy. Of the 13 cases, only 1 had multi-locus lesion. The average tumor size was (6.07 ± 1.34) cm. The lymph node involvement was found in 46.2% of the patients. Local and distant failure occurred in 50% and 30% patients, respectively. The most common site for dissemination was the lung. Four cases died within 3 years after initial operation. The 1-, 2-, 5-, 10-year overall survival rates of the 11 cases were 72.7%, 54.5%, 27.3% and 9.1%, respectively, with a median survival time of 27 months. Epithelioid sarcoma is a rare disease. The prognosis for patients with epithelioid sarcoma is poor because of a high propensity for local recurrence, lymph node metastases, and/or distant metastases. The definite diagnosis depends mainly on the pathologic examination. Wide surgical excision is the mainstay treatment, and radiation and chemotherapy have been used occasionally as adjuvant therapy but have had limited success.