Clear cell sarcoma (malignant melanoma) of soft parts - A clinicopathologic study of 30 cases

BACKGROUND, Clear cell sarcoma, or malignant melanoma of soft parts, is a r are tumor that occurs predominantly in the extremities of young adults. The importance of surgery has been well established. However, the role of adju vant radiotherapy has yet to be determined. METHODS, Thirty cases of clear cell sarcoma that occurred in The Netherland s between 1978 and 1992 were studied retrospectively. Follow-up information on 29 patients was obtained; the follow-up period ranged from 4 to 241 mon ths, with a median of 30 months. AU tumors occurred in the extremities, mos tly of young adults. RESULTS. The 5-year survival rate of the 29 patients was 54%. For the 23 pa tients who presented with localized disease, the 5-year survival and 5-year disease free survival were 65%. Eleven of these patients remained disease free after resection of the primary tumor. Patients with a tumor 2 cm or sm aller had better survival than patients with a larger but still-localized t umor (P = 0.009). Adjuvant radiotherapy to the primary tumor site also seem ed to have a beneficial effect on survival (P = 0.036). AU patients with a local recurrence (8 patients) or regional lymph node metastasis (13 patient s) developed distant metastasis. Fourteen of 18 patients with distant sprea d died of their disease; 2 patients were still alive with disease and 2 pat ients were disease free, 7 and 32 months after resection of solitary distan t metastases. CONCLUSIONS. Early diagnosis and initial radical surgery are essential for a favorable outcome. Once regional lymph node metastasis or hematogenous di ssemination has occurred, the prognosis is dismal. (C) 1999 American Cancer Society.