A Case of Intrahepatic Cholangiocarcinoma Arising 26 Years after Excision of Congenital Biliary Dilatation

Abstract

We report a case of intrahepatic cholangiocarcinoma arising 26 years after excision of congenital biliary dilatation (CBD). A 46-year-old man with epigastralgia and back pain admitted for liver tumor and stenosis of the hilar bile duct had previously undergone reconstruction by Roux-en-Y hepaticojejunostomy for CBD in 1975. Computed tomography showed a liver tumor 30 mm in diameter on S4 and lymph node swelling surrounding the hepatoduodenal ligament. Endoscopic retrograde cholangiopancreatography visualized pancreaticobiliary maljunction and multiple stones in the residual intrapancreatic bile duct. Percutaneous transhepatic cholangiodrainage was initiated when jaundice progressed, and fistelography recognized bile duct stenosis of the anterior, posterior, and caudate branches. Angiography showed stenosis of the left hepatic artery and left branch of the portal vein. We diagnosed intrahepatic cholangiocarcinoma. Extended left lobectomy and caudate lobe resection were scheduled, but we undertook gastrojejunostomy due to liver metastasis and invasion of the hepatoduodenal ligament. The patient died of liver failure 1 month later.