Am J Clin Pathol 1981 Aug;76(2):172-7

Hepatoma in siblings with progressive familial cholestatic cirrhosis of
childhood

Ugarte N, Gonzalez-Crussi F

Abstract
This report describes four siblings affected with familial intrahepatic
cholestasis detected in early infancy. In the two male siblings, biliary
cirrhosis and fatal hepatocellular carcinoma later developed, whereas the female
siblings have had persistent hepatomegaly and recurrent episodes of cholestasis.
Sequential biopsies show that this rare disorder of unknown etiology must be
added to the many causes of giant cell transformation of the liver in infancy.
Its oncogenic risk, particularly in males, has not been generally appreciated