Cholangiocarcinoma in a patient with biliary cysts

A 15-year-old girl with multiple intra- and extrahepatic biliary cysts is presented. At a primary laparotomy a massive dilatation of the choledochal duct was found and a choledochocysto-duodenostomy was made. Initially the patient recovered but 1 1/2 years later a mass reappeared under the right costal arch. At a second laparotomy multiple neoplastic changes were found on the right and left liver lobes. Histologic examination of biopsies from the liver showed the picture of a cholangiocarcinoma.