Primary Biliary Cystic Tumors of the Liver

Five cases of primary cystic tumors of the intrahepatic bile duct are documented over a 5-yr period. Clinically, two cases had recurrent episodes and one had a first attack of jaundice, fever, and pain in the upper abdomen; one complained of abdominal fullness and one was asymptomatic. The appearance of the tumors were single, large, multilocular, and cystic. Important radiological findings included irregular thickness of the cystic wall, internal septation, and papillary projection. Marked biliary dilation was demonstrated in four of the patients. On the cholangiogram, amorphous filling defects were seen in the dilated extrahepatic bile ducts, and mucinous material could be removed from the ducts as well. Obstruction of the bile ducts by this mucinous bile was assumed to be the cause of cholangitis in three patients. Histological examination revealed three cases of cystadenoma and two cases of cystadenocarcinoma.