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Twenty-two pediatric patients with a stage IV-A or IV-B neuroblastoma have been treated with multimodal therapy consisting of intensive chemotherapy and surgery at Niigata University Hospital since 1985. The patients first received six cyclic courses of intensive chemotherapy ［regimen A_1］, consisting of cyclophosphamide ［1,200mg/m^2］, vincristine ［1.5mg/m^2], tetrahydropyranyl adriamycin ［pyrarubicin 40mg/m^2］, and cisplatin ［90mg/m^2］. Primary tumors and involved regional lymph nodes were removed sometime during first six cycles of chemotherapy. After the six cycles of ［Regiment A_1］, patients received alternating treatment with ［Regimen A_2］consisting of cyclophophamide ［1,500mg/m^2］, THP-adriamycin ［50mg/m^2］, cisplatin ［90mg/m^2］, and ［Regimen C］consisting of cyclophosphamide ［1,500mg/m^2］, DTIC ［250mg/m^2］. Two-year survival rate was 59%. Nine patients have now survived with disease free at 2 years after initiation of the treatment （survival rate: 41%）. From the viewpoint of prognosis, the influencing factors are as follows: small round cell type, diploidy, VMA（－）, NSE more than 100ng/ml, N-myc over 8 times of normal value.

Neuroblastoma is one of the malignant tumors in chidren. In Oct, 1985, mass-screening of 6-month-old infants for this disease started in Niigata Prefecture. Up to now, this screening test has detected 17 neuroblastoma patients among apparently normal infants. All of the 17 patients received early treatment, and have been alive and well. However, besides these patients, additional 17 patients were discovered with this disease which manifested itself at an older age than those found by the screening test. Age-related incidence of the 17 patients was 4 cases under 1year of age, 5 at 1year, 3 at 2 yrs, 3 at 3 yrs, 1 at 4 yrs, 1 at 5 yrs, respectively. They were found at a later stage of the disease than those found on the routine screening test. Despite the normal judgement of mass-screening test performed at the age of 6 months, 7 of 15 cases (46.5%) were found to have increased urinary excretion of VMA and/or HVA. Although the patients were treated vigorously for advanced neuroblastoma, 8 (47%) of the 17 patients had already died, and 3 cases have been on relapses. These data, revealing poor prognosis for the patients found at an older age, prompted us to screen children at 1 year and 6 months of age, using VMA test on dried urine-soaked paper. Between Sept 1992 and Feb 1993, 1,428 (62.5%) of 2,172 children whose parents had been informed the significance of the test, received the screening test in Niigata City. However, probably due to rather small number of the samples, we still have not found any of neuroblastoma patients in this preliminary test. From these results, and considering the poor prognosis of the patients with advanced neuroblastoma, we propose a second mass-screening test of young children for neuroblastoma at the age of 1year and 6 months.

We describe the results of infantile mass-screening test for neuroblastomas for 5 years in Niigata prefecture which started in 1985. Since Oct 1985, 112, 965 infants, aged 6～8 months, have been screened for elevation of urinary VMA level using urine spotted-filterpaper. During the five years nine children (1/12, 552) were found to have neuroblastomas with varying stages II(4 cases), III(1), and IV(4). They were operated on, and had subsequent chemotherapy combined with or without radiation. All the patients are alive and well with no relapses. Other than the 9 patients, 8 children, aged 6 months to 2 years, developed neuroblastomas during the five years. Urinary VMA excretions were elevated in 3 of them, but normal in 3, and remaining two patients had not received the mass-screening test. These results demonstrate that the screening programe for neuroblastomas in Niigata prefecture was successful and useful for the early detection and treatment of neuroblastomas.

92 patients with acute lymphocytic leukemia （ALL）, 29 patients with acute nonlymphocytic leukemia （ANLL）, and 6 patients with chronic myelogenous leukemia （CML） admitted to the Department of Pediatrics, Niigata Cancer Center Hospital between Jan. 1961 and Dec. 1988 are reviewed in this study. From 1981 to 1985, a complete remission in 96% of patients with ALL could be archived by remission induction therapies according to risk factors. The rate of continuous complete remission was 74% at 3 years and 57% at 5 years after a complete remission of the bone marrow. Cessation of maintenance therapies in 21 patients with ALL, who began remission induction therapies from 1977 to 1985 and continuous complete remission for more than 3 years, was undertaken. After cessation of therapies, the rate of continuous complete remission was 86% at 3 years and relapses were not seen for more than 3 years. Relapses occured in 4 patients from 2 to 26 months after cessation of therapies. The sites of the relapses were 3 case of bone marrow, and 1 case of bone marrow/CNS. Compared with ALL, the results of remission induction and maintenance therapies in childhood ANLL and CML are not as positive and bone marrow transplantation must be introduced in pediatrics.

In Niigata prefecture, we have screened 27,894 infants at the age of 6 months for neuroblastomas using urine-spotted filterpapaer. 1,121 infants (4.0%) were positive for urinary vanyl mandelic acid (VMA), and 94 infants (0.3%) were referred to our hospital for further examination. Various clinical tests including determination of urinary cathecholamines on high-power liquid chromatography, abdominal echography, intravenous pyelography, CT-scanning, bone scintigraphy, and blood chemical analysis were performed, which revealed no abnormal findings in the infant suspected to have neuroblastomas.