SOFT-TISSUE SARCOMA OF THE HAND IN CHILDREN - CLINICAL OUTCOME AND MANAGEMENT

Soft tissue sarcomas (STS) of the hand are rare in children and adoles cents. From 1965 through 1995, 18 children with STS of the hand were t reated at our institution. Rhabdomyosarcoma (RMS) was diagnosed in 11 patients; alveolar histological results predominated (7 of 11 cases). Seven patients presented with metastatic disease and died 4 to 23 mont hs (median, 9 months) from diagnosis; their surgical treatment compris ed above-elbow amputation (n = 1), local excision (n = 1), and biopsy (n = 5). For the four patients who presented with localized RMS, surge ry consisted of wide local excision (n = 1), local excision (n = 2), o r ray amputation (9 = 1). With an average follow-up of 5.5 years (rang e, 4 months to 18 years), 3 of the 11 patients diagnosed with RMS stil l survive (27%). The remaining Seven patients presented with nonrhabdo myosarcomatous soft tissue sarcoma (NRSTS); the most common histologic al variants were epithelioid and malignant fibrous histiocytoma (two c ases each). Surgical treatment for these patients comprised ray amputa tion (n = 3), wide local excision (9 = 3), excisional biopsy in = 1), and regional lymph node dissection in = 3). One patient received adjuv ant multiagent chemotherapy; three patients received supplemental radi otherapy. Six of the seven (85%) patients are alive with no evidence o f disease at an average follow-up of 4.7 years (range, 6 months to 12 years). Copyright (C) 1997 by W.B. Saunders Company.