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The courses of 18 children with neurogenic sarcoma (NS) treated at the Children's Hospital of Philadelphia between 1958 and 1979 were reviewed. Their median age was 11 years (range, 0.3–16 years); 12 were female and six male. Eleven children had stigmata of neurofibromatosis (NF). One patient had two separate tumors simultaneously. Nine of the tumors were primary in an extremity; six were intraabdominal, two craniofacial, and two truncal. Eleven of the 18 children had grossly complete surgical removal of the primary tumor, whereas seven had gross residual disease after surgery; none had distant metastases detected at diagnosis. The patients were divided into two groups according to whether treatment was primarily surgical (group A) or multimodal (group B). Treatment for group B patients included surgery, local radiotherapy, and multiple-agent chemotherapy. Two of 12 patients (17%) treated primarily with surgery are surviving four and 14 years later with no evidence of recurrent disease (NED); both had complete removal of the primary NS. Two additional group A patients relapsed but were retrieved with radiotherapy and multiple-agent chemotherapy. The other eight patients in group A died of tumor at a median of 14 months from diagnosis. In contrast, five of the six group B patients (83%) treated with adequate multimodal therapy (at least 4,000 rads local radiation plus vincristine, actinomycin D, and cyclophosphamide ± Adriamycin) are relapse-free and surviving at a median of 27+ months from diagnosis. Nine of the 18 patients are free of disease, four with and five without NF. The results suggest that multimodal therapy similar to that for rhabdomyosarcoma should be used for children with neurogenic sarcoma.

We evaluated the intensity of the periodic acid-Schiff (PAS) stain reaction by examining 200 lymphoblasts from the initial marrow aspirate specimen of 38 children with acute lymphoblastic leukemia (ALL). The patients were separated into low, intermediate, and high groups according to mean PAS score. The groups were then compared in terms of age, sex, initial hematologic values, T- and B-lymphocyte markers, percentage of patients in remission six months after diagnosis, and percentage surviving. Significant differences were found between the low group and the other two groups with respect to sex, height of initial white blood cell (WBC) count, outlook for continued remission at six months, and overall survival (P < 0.05). The low-score patients were predominantly males, whereas the other PAS-score groups contained approximately equal numbers of males and females. The low-score patients had a higher median initial WBC than the others, but all three groups had a similar proportion of patients with initial WBC under 20,000/cu mm. Low-score patients were less likely to be in remission six months from diagnosis, and they had shorter survival than the intermediate- and high-score patients (P < 0.05). The results suggest that children with ALL whose lymphoblasts stain weakly with PAS have a worse prognosis than those with intermediate or high PAS reactivity. PAS reactivity may vary independently of the WBC at diagnosis in children with ALL, because children with a low PAS score have a poor outlook even with initial WBC under 20,000/cu mm.