Hepatoblastoma in infant sisters

A family in which 2 infant sisters died with hepatoblastoma, the first reported familial occurrence of this neoplasm, is presented. The children had no heritable or congenital disorders known to increase the risk of hepatoma, and laboratory studies revealed no clues to pathogenesis. The detection of serum alphafetoprotein in the proband suggests that the test may be useful in the diagnosis of hepatoblastoma as well as hepatocellular carcinoma, and in the screening of children predisposed to these neoplasms. Sibship aggregation has also been observed with adrenocortical neoplasia and Wilms' tumor, which share with hepatoblastoma an association with certain congenital malformations.