One-hundred-eighty-eight children up to 16 years of age were randomized in the second National Wilms' Tumor Study (NWTS) with tumors that were confined to the kidney and that had been totally excised (Group I). Most fared well whether treated for six or for 15 months with both actinomycin D (AMD) and vincristine (VCR). No postoperative radiation therapy (RT) was given. The two-year relapse-free survival (RFS) and two-year survival rates were 88 and 95%, respectively. Two-hundred-sixty-eight randomized patients with more advanced local lesions (Groups II and III) and 57 with distant metastases (Group IV) had postoperative RT and were scheduled for 15 months treatment with either AMD and VCR (Reg. C) or AMD plus VCR plus Adriamycin (Reg. D). The 77% two-year RFS rate for Reg. D was significantly different from the 63% with Reg. C. As in the first NWTS, patients with tumors of unfavorable histology (UH) had a significantly worse prognosis than those with favorable histology (FH), as did those with positive nodes. Survival rates at two years were 54% for UH vs. 90% for FH, and 54% vs. 82% for those with and without lymph node involvement.

The National Wilms' Tumor Study, initiated in 1969, tested competing treatment strategems for patients with tumors ranging from Group (Gp) I (tumors confined to the kidney and totally removed) to Gp IV (remote metastases present at diagnosis). Three hundred and fifty‐nine of 606 registered patients were randomized in the trial. Gp I patients under 2 years of age fared well whether postoperative radiation therapy (RT) was or was not added to 15 months' maintenance actinomycin D (AMD). Their prognosis was better than that for older cohorts similarly treated, in whom the difference in relapse rates between treatment groups were suggestive of an RT effect. Combined AMD and vincristine (VCR) gave better results than either agent alone in patients with more advanced tumors (Gps II and III) still confined to the abdomen, all of whom received postoperative RT as well. Preoperative VCR given Gp IV patients in addition to postoperative RT, AMD, and VCR did not improve results. The frequency of mesoblastic nephroma (1%), of bilateral tumors (5%), and of incorrect preoperative diagnosis of Wilms' tumor (5%), the toxicities of the various regimens, and other ancillary data are presented and discussed.

Four hundred and twenty-three children with newly diagnosed rhabdomyosarcoma have been entered to date in the Intergroup Rhabdomyosarcoma Study (IRS), which began in 1972. Patients were classified into Clinical Disease Groups (Stages) I-IV, based on disease extent and resectability, and treatment regimens were randomly assigned according to group. Two hundred and seventy-eight of 423 patients are evaluable for this analysis. Thus far, for Group I disease (localized/completely resected), disease control achieved by vincristine, dactinomycin, and cyclophosphamide (VAC) given in combination for 2 years has not been enhanced by the administration of postoperative radiation to the tumor bed. To date, 92% of patients in both irradiated and nonirradiated groups exhibit no evidence of disease, and 92-96% are still alive, with the median time of follow-up being 72 weeks. For Group I1 disease (microscopic residual/nodal involvement), VAC given for 2 years has not been found to be more effective than vincristine plus dactinomycin given for 1 year, both groups also having received postoperative irradiation. Thus far, over 85% of patients on either treatment have no evidence of disease and 90% are still alive. Survival and complete remission durations range from 1+ to 143+ weeks and the median duration of follow-up is 45 weeks. Chemotherapy as initial treatment has been studied in Group 111 (localized sarcoma not treated initially by gross total resection) and Group IV (metastases present at diagnosis) patients. They have received either intensive "pulse" VAC or "pulse" VAC plus Adriamycin, and radiation has been administered after 6 weeks. Eighty-one percent of patients in Group 111 and 81 -83% in Group IV have responded favorably, with complete regression of disease having been observed in over one-fourth of patients even before the start of radiation and in approximately one-half of all the patients after receiving radiation therapy. There is no indication as yet that one treatment regimen is superior to the other. Seventy-nine percent of patients in Group 111 are still alive (0+ to 154+ weeks) and 69% remain in continuous response (0+ to 139+ weeks) with the median duration of follow-up being 41-44 weeks. Fifty percent of patients in Group IV are still alive ( O f to 127+ weeks) with a median time of follow-up of 41-44 weeks. Tumors arising either from genitourinary sites or the extremities have had a higher incidence of lymphatic spread than tumors in all other primary sites of origin.CUWC~

A total of 105 patients with advanced acute leukemia in relapse received 123 trials of L‐asparaginase. Three different schedules were used, two of which involved simultaneous administration of vincristine and prednisone. Treatment was temporarily interrupted because of toxicity in 14 patients, and permanently discontinued in an additional 31. In 13 patients, this occurred before 10 doses of L‐asparaginase were given, while in the remaining 18 patients, therapy was stopped after remission was attained. The major toxicities were pancreatitis (fatal in four patients), hypersensitivity reactions, disturbances of liver function (fatal in two patients), and clinical and laboratory manifestations of central nervous system (CNS) dysfunction. Diabetic keto‐acidosis was encountered in two patients and was fatal in one. Severe leukopenia ascribed to L‐asparaginase occurred in two patients, in one of whom it was the contributory cause of death. Toxicity was not clearly different with any of the three treatment schedules. Most of the patients with only laboratory evidence of toxicity had no associated clinical manifestations, and this did not appear to decrease the likelihood of obtaining a remission.

Survival data on 78 children with rhabdomyosarcoma indicated that age, histology, primary site, extent of disease, and treatment each had a significant influence on prognosis. The best survival occurred in patients with sarcoma botryoides, the poorest with alveolar rhabdomyosarcoma, and the intermediate with embryonal rhabdomyosarcoma. Tumors of the orbit had the best prognosis and tumors of other structures of the head and neck had the worst prognosis. Children under 7 years of age had better survival curves than those older than 7 years. The median duration of survival for the entire group was 19.2 months (5‐year survival rate of 35%). The median duration of survival among 54 children with localized disease was 53.3 months; none of the 24 children with extensive or metastatic disease at diagnosis survived as long as 30 months.