EPITHELIOID SARCOMA IN CHILDREN

Literature regarding the natural history and prognosis of epithelioid sarcoma in the pediatric population is sparse. During the past 11 year s, eight patients with epithelioid sarcoma were treated at St Jude Chi ldren's Research Hospital. Of the eight children, six of whom were mal e, seven presented with localized disease and one with metastatic dise ase. The primary tumor was located in the upper extremity in four pati ents; the other sites were the scalp, external ear, back, and mediasti num. The mean duration of symptoms before diagnosis was 8.3 months (ra nge, 1 to 12 months). Surgical treatment comprising amputation or wide local excision was performed in six patients, three of whom had addit ional lymph node dissection. Three patients received multiagent chemot herapy, and four patients were treated with supplementary radiation. T wo patients died of progressive local and distant disease within 9 mon ths of diagnosis. The other six patients are alive and have no evidenc e of disease 12 months to 10 years 6 months after the completion of tr eatment. Copyright (C) 1996 by W.B. Saunders Company.