Problems of neuroblastoma screening for 6 month olds and results of second screening for 18 month olds.

Nationwide neuroblastoma mass screening for 6-month-old infants (first screening) was introduced in Japan in 1985. About 110 neuroblastoma cases are detected annually by the first screening and treated, with a survival rate of 97%. Sensitivity of the first screening (positive cases/positive cases+false negative cases) is about 75%, and the prognosis of false-negative cases is unfavorable. A second screening at 18 months of age was started to rescue false-negative cases in Miyagi Prefecture in May 1992. Of 62 neuroblastoma cases treated in our hospital since 1985, 40 cases had received the first screening. Twenty cases were positive at first screening, 18 cases were false negative, and 2 cases were false negative and picked up by the second screening. Age distribution of false-negative cases ranged from 12 to 83 months and included 12 cases younger than 36 months old. Only 5 of 18 false-negative cases are alive without the disease. From May 1992 to November 1993, 14,282 infants had received the second screening (compliance rate: about 75%), and 2 neuroblastoma cases were detected. The first case was stage III with paraortic lymph node metastases, Shimada UH, aneuploidy and negative N-myc amplification. The second case was stage II with Shimada FH, aneuploidy, and negative N-myc amplification. Both cases are alive now without the disease after undergoing radical operation and chemotherapy. The first screening is effective for early detection of neuroblastoma cases, but the sensitivity is insufficient; the authors recommend a second screening to rescue false-negative cases.

Surgical treatment of congenital dilatation of the bile duct with special reference to late complications after total excisional operation.

The surgical treatment of 100 cases with congenital dilatation of bile duct with special reference to late complications was analyzed. There were no deaths nor occurrences of malignancy. Among 91 patients who had undergone the standard operation, namely total excision of the dilated extrahepatic bile duct and reconstruction after Roux-en-Y hepaticojejunostomy, there were one early complication (pancreatic juice leakage) and five late complications (four intrahepatic gallstones and one liver abscess). The cause of intrahepatic gallstone formation after a total excisional operation was attributed to the remaining intrahepatic bile duct dilatation and the stenosis located between the intrahepatic bile duct dilatation and the common hepatic duct. Accordingly, these results support the total excisional procedure for this condition; however, with regard to the cases associated with cystic dilatation of intrahepatic bile ducts, completely free bile drainage from the dilated intrahepatic biliary system should be performed at the radical operation.

Effect of VAB-6 combination chemotherapy on malignant germ cell tumours in childhood.

The VAB-6 protocol was applied to 5 children with malignant germ cell tumour. Four of them achieved complete remission after 3 inductions of this protocol and surgery. Death in one case was attributed to chemotherapy. Complete responders have been in remission now for 4 months to 2 years without maintenance chemotherapy. Adverse effects including myelosuppression, ototoxicity, cardiotoxicity and pulmonary toxicity were observed. The VAB-6 protocol is an effective chemotherapy in children with malignant germ cell tumour and requires only 3 to 4 months of treatment.

Persistent cervical thymus with a small cyst in a newborn infant.

A boy, aged 1 month, who showed a tumour mass in the right neck, is presented. Operation disclosed that the tumour was a cervical thymus with a small cyst. The cervical thymus degenerated gradually into hypoplasia or thymic cyst due to the abnormal descent of the thymus. It is rare that cervical thymus and/or thymic cyst is detected before the age of one year.