Intrahepatic cholangiocarcinoma arising in Caroli's disease.

Abstract

Caroli's disease (CD) is a rare congenital disorder characterized by non-obstructive segmental saccular dilatation of the large intrahepatic bile ducts.1 Two forms of CD have been described. The pure type is characterized by cystic dilatation of the intrahepatic bile ducts, while the complex type - also designated Caroli's syndrome - is associated with congenital hepatic fibrosis (CHF) and autosomal recessive polycystic kidney disease.2,3,4 CD usually affects the liver diffusely but some localized forms (monolobar CD) affect only a single lobe, predominantly the left lobe.5,6 Complications of Caroli's disease are frequently related to the stagnation of bile resulting from the saccular dilatation of bile ducts, and include intrahepatic lithiasis, recurrent cholangitis, abscess formation, septicemia and amyloidosis. Patients with CD have also been shown to be at risk for intrahepatic cholangiocarcinoma.7,8 A case of intrahepatic cholangiocarcinoma arising in a localized CD is described and the recent literature discussed.