Abstract
24 patients (pts) aged 3 mo.-16 yr. (median, 12 yr.) were treated for NGS at the CHCRC from 1958 through 1984. 16 pts (67%) had neurofibromatosis (NF). The tumors arose in an extremity or the trunk (14 pts), retroperitoneum-pelvis (6 pts), or other sites (4 pts). 13 pts underwent grossly complete excision of localized sarcoma; of them, 7 had no known residual tumor and 6 had microscopic residual. The other 11 pts had visible residual localized (10 pts) or metastatic sarcoma (1 pt). 8 pts were treated on a protocol with radiation therapy (4000-6000 rad) and VAC (vincristine, actinomycin D, cyclophosphamide) ± Adriamycin. The other 16 pts were treated variably.
The proportion of pts continuously tumor-free and alive is:
Five of 8 protocol pts are tumor-free survivors (TFS), compared to 4 of 16 non-protocol pts. Surgical removal of all gross tumor is important: 8 of 13 pts with excision are TFS compared to 1 of 11 with gross residual sarcoma. Pts with NF fare poorly: only 3 of 16 are TFS compared to 6 of 8 without NF. We conclude that a more effective treatment program is needed for children with NGS, especially for those with NF.
Supported in part by USPHS Grants CA-19372 and CA-14489.
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Raney, B., Schnaufer, L., Ziegler, M. et al. 932 MULTIMODAL TREATMENT OF CHILDHOOD NEUROGENIC SARCOMA (NGS). Pediatr Res 19, 266 (1985). https://doi.org/10.1203/00006450-198504000-00962
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DOI: https://doi.org/10.1203/00006450-198504000-00962