Soft-tissue sarcomas other than rhabdomyosarcoma in children.

Forty-seven children with nonrhabdomyosarcomatous soft-tissue sarcomas (NRSTS) were treated by the Hematology-Oncology Service at Texas Children's Hospital, Houston, Texas, between 1958 and 1990. The male:female ratio was 1:1, and the median age was 11 years (3 weeks-16 years). A preexisting condition was found in 9/47 (19%) patients including neurofibromatosis (3), Down's syndrome (1), spina bifida (1), congenital facial asymmetry (1), giant pigmented nevus (1), juvenile onset diabetes mellitus (1), and acquired immune deficiency syndrome (1). The site of primary tumor was head and neck (3), trunk (33), and extremities (11). Twenty-four patients (51%) have survived free of disease with a median follow-up of 5 years (4 months-22 years). No patient whose disease recurred achieved a second remission. Of the 19 patients with group I disease, 16 (84%) survived free of disease. Wide excision of the primary tumor, with no microscopic residual disease, was associated with the greatest chance of disease-free survival.