Epithelioid sarcoma of the hand

Twenty-eight patients were treated for a primary epithelioid sarcoma of the hand. Twenty-seven patients (96%) had excisions before evaluation, including 11 (39%) with multiple prior excisions with varying diagnoses before epithelioid sarcoma, and all had surgical treatment after referral. The patients' surgical management included three patients with amputation at the forearm, three patients with wide excision, and 21 patients with a partial amputation of the hand. The followup period averaged 120 months (range, 24-276 months). Eighteen patients have no evidence of disease at last followup. Treatment failures included one local recurrence, four regional metastases, and five distant metastases. Five patients died secondary to disease. Two patients are alive with disease, and three are alive with no evidence of disease after additional treatment. After aggressive surgical management with negative margins, 71% of the patients were alive without evidence of disease at the last followup, with a 5- and 10-year survivorship of 85%. Our goal is to review: (1) the effectiveness of preoperative imaging, (2) the role of adjuvant therapy, (3) survival after alternative resections, and (4) function after resection. Level of Evidence: Therapeutic study, Level IV (case series-no, or historical control group).