Radiation therapy of epithelioid sarcoma.

Epithelioid sarcoma is an uncommon tumor characterized by its appearance in the distal extremities (especially the forearm, wrist, and ankle), the tendency to recur locally, and high probability of spread to regional lymph nodes. Most patients have been treated surgically, and the potential role of radiation therapy alone or combined with surgery is not well defined. The authors treated eight patients with epithelioid sarcoma. Four patients were male; patients ranged in age from 7 to 66 years (median, 54.5). Six patients had upper extremity lesions, and two had lower extremity lesions. Median maximum tumor dimension was 3.0 cm. Three patients had Grade 2 lesions, and the others had grade 3 lesions. Regional lymph nodes were involved initially or during the course of the disease in five of our eight patients (62%). Five patients were treated following surgery for primary or recurrent tumor, with a median dose of 68 Gy (TDF 109). Four were NED with follow-up at 18, 24, and 84 months, respectively, and the other patient failed locally and in regional nodes at 6 months (treated successfully by salvage amputation) and is now living at 96 months with distant metastases. One patient treated pre-amputation with 50 Gy (TDF 81) is NED at 12 months. Two patients received radiation therapy alone, with a median dose of 65 Gy (TDF 104). One patient with a 22-cm primary died with persistent local disease as well as metastases, and the other died with distant metastases but with local control at 18 months. Four patients with local control did not undergo amputation, and all have unimpaired function of the affected extremity. Local control and survival for this group of patients is similar to that for other patients with upper extremity or high grade sarcomas. The authors concluded that radiation combined with surgery achieves a low rate of local recurrence, and a high likelihood of maintaining a functional extremity and good cosmesis.