Primary Carcinoma of the Liver in Childhood: An Epidemiologic Study

Abstract

In a search for etiologic clues, study was made of 282 death certificates of U.S. children who died of hepatic carcinoma, 1960–1964, and of 76 hospital charts of children with this neoplasm. There was an early peak in mortality which, coupled with the occurrence of neonatal liver cancer, suggests that in early childhood the tumor has a prenatal origin. The array of congenital defects recorded in the hospital series indicates that this tumor may share etiologic mechanisms with Wilms' tumor and adrenocortical neoplasms. Two children with liver cancer had congenital hemihypertrophy and two had extensive hemangiomas, anomalies which occur in excess with Wilms' tumor and adrenocortical neoplasms; one child had Wilms' tumor synchronous with the diagnosis of liver neoplasia and another had agenesis of the adrenal gland, a defect observed with adrenocortical tumors. In addition, 5 children had antecedent liver diseases: nonspecific cirrhosis (2 cases), neonatal hepatitis, congenital biliary atresia, and de Toni-Fanconi syndrome (1 case each). Additional cases found in the mortality series included von Gierke's disease. The early peak incidence of liver neoplasia seems related to coexisting congenital anomalies such as hemihypertrophy and to liver damage following biliary atresia or neonatal hepatitis, whereas liver disease due to nonspecific or metabolic cirrhosis is a precursor to neoplasia in older children and adults. Geographic variation in liver cancer mortality occurred in children under 5 years of age, which suggests an environmental influence on the early peak. No evidence was found to implicate genetic factors, other than their role in certain disorders which increase the risk of liver cancer.