Association of carcinoma with congenital cystic conditions of the liver and bile ducts.

Reported cases of carcinoma arising in association with congenital cystic conditions of the liver and bile ducts are tabulated with respect to the specific type of congenital abnormality. The occurrence of carcinoma in solitary nonparasitic cysts of the liver or as a complication of polycystic liver disease is distinctly rare. Carcinoma will arise with a frequency of approximately 1% in congenital hepatic fibrosis, 4% in choledochal cyst and 7% in congenital cystic dilatation of the intrahepatic bile ducts. Those congenital conditions exposing the epithelium to the bile directly are at greater risk for the development of malignancy. Carcinoma in this clinical setting occurs several decades earlier than otherwise expected.