The role of chemotherapy in childhood soft tissue sarcomas other than rhabdomyosarcomas: Experience of the Northern Israel Oncology Center

Forty-three children with nonrhabdomyosarcomatous soft tissue sarcomas (NRS TS) were treated at the Northern Israel Oncology Center in Haifa, Israel, f rom 1971 to 1996. The male:female ratio was 1.5:1 and the median age of pat ient was 10 years (range, 3 months-18 years). The most common histopatholog ic diagnoses were fibrosarcoma (32.5%) and synovial sarcoma (16%). The site s of primary tumor were lower limb (35%), trunk (18%), upper limb (16%), he ad and neck (16%), and retroperitoneum (11%). By Intergroup Rhabdomyosarcom a Study classifications, 13 patients presented as group 1, 15 patients as g roup 11, 10 patients as group III, and 5 patients as group IV. Median follo w-up time was 63 months (range, 6 months-18 years). The estimated survival after a 5-year period is 72% (SE +/- 17) for patients in group I, 75 +/- 15 % in group II, 90 +/- 9% for patients in group III, and 40 +/- 21% for pati ents in group IV. Eleven patients relapsed; 4/6 who developed local relapse were cured and are alive with disease, while 4/5 who developed distant met astases are dead. For the 28 patients who underwent complete resection at d iagnosis, the estimated survival after a 5-year period is 87 +/- 5% vs. 60 +/- 17% for the 15 patients who Underwent partial excision or biopsy. Local radiotherapy was delivered after surgery to group III patients. Preoperati ve and postoperative chemotherapy was delivered to the patients of groups I II and IV and postoperative chemotherapy only to group II patients. Chemoth erapy produced demonstrable gain in survival for group II and III patients but not for patients with metastases. The authors conclude that an aggressi ve surgical approach is needed in patients with NRSTS. Chemotherapy may hel p as a preoperative treatment in bulky disease or as a postoperative treatm ent for microscopic residual disease.