Alpha-fetoprotein-producing clear cell carcinoma of the extrahepatic bile ducts.

A 79-year-old woman visiting our hospital with chief complaints of epigastric pain and jaundice was emergently admitted. Her alpha-fetoprotein (AFP) level was as high as 2265 ng/mL at admission. Her abdominal computed tomography scan revealed dilation of the intrahepatic bile duct and a tumorlike lesion protruding into the cystic duct and gallbladder from the junction between the middle portion of the bile duct and the right and left hepatic ducts. Surgery revealed a tumor extending from the extrahepatic bile duct (EHBD) to the cystic duct, with no intrahepatic tumor components. The tumor was histologically diagnosed as an AFP-producing cholangiocarcinoma of the clear cell type, originating from the EHBD. None of the previously reported cholangiocarcinomas of the AFP-producing clear cell type have been confined to the EHBD or have been resectable in a curative manner without hepatectomy.