A Case of Perihilar Cholangiocarcinoma Occurring 49 Years after Surgery for Congenital Biliary Duct Dilatation and 28 Years after Pancreaticoduodenectomy for Pancreatic Duct Stones

Abstract

A 50-year old woman had been diagnosed with congenital biliary dilatation and underwent choledochojejunostomy with extrahepatic bile duct resection at the age of 1 year 11 months. When she was 22 years old, pylorus-preserving pancreaticoduodenectomy was performed for pancreatic duct stones. At the age of 49 years, she visited our hospital because of repeated acute cholangitis. CT demonstrated dilatation of the jejunal limb and subsequent stenosis near the choledochojejunostomy site. Endoscopy revealed a granular mucosal pattern in the perihilar bile duct, which spread widely beyond the secondary branch of both the right and left hepatic ducts and invaded the jejunal limb. The histological diagnosis was adenocarcinoma based upon biopsy of the granular mucosa of the right and left hepatic bile ducts and perihilar bile ducts, and we clinically diagnosed the case as unresectable perihilar cholangiocarcinoma, T4aN1M0, stage IVA. The patient underwent jejunal bypass between the dilated jejunal limb and the intact jejunum for recurrent cholangitis. She was treated with systemic chemotherapy, and died 12 months after the surgery. In congenital biliary duct dilatation, many reports discuss bile duct cancer even after extrahepatic bile duct resection with choledochojejunostomy. Ours was a rare case in which bile duct cancer occurred after pancreatic juice flowed into the perihilar and hepatic bile ducts again following pancreaticoduodenectomy, even though pancreatic juice flowing into the ducts was avoided from 1 year to 22 years of age.