Twenty-six cases of epithelioid sarcoma having a minimum of 5 years follow-up are presented. The patients ranged in age from 11 to 64 years; 18 were between 20 and 40 years of age. Males predominated (18:8). The most common tumor locations were the fingers (6 cases), the wrist (5 cases), and the hand (4 cases). All tumors were typical histologically, with medium-sized to large epithelioid and plump spindle cells, a predominantly nodular growth pattern, and intranodular collagen. Features seen in a minority of cases included hyalinized collagen, calcification, myxoid areas, and focal small cells. The major factor related to distant metastasis and death from tumor proved to be tumor size; all 7 patients with neoplasms > or = 5 cm died of tumor, and 6 had distant metastases, whereas only 2 of 10 patients with smaller tumors developed distant metastases and died (of patients with neoplasms of unknown size, 3 died of tumor and 2 of those along with 1 other had distant metastases). Five patients had regional lymph nodes involved (4 with tumors > or = 5 cm, 1 with tumor of unknown size), and all died. Local recurrence was primarily associated with treatment by excision alone (9 of 10 cases) rather than amputation (2 of 8 cases) or excision and radiation (2 of 8 cases); recurrences were often controlled by subsequent amputation. When tumor size and treatment were taken into account, histological variables including mitotic rate, tumor necrosis, and perineural invasion were not significantly related to recurrence, metastasis, or patient survival.