A case of bile duct cancer, 43 years after cholangiojejunostomy for congenital biliary dilatation

Abstract

We recently encountered a patient who developed bile duct cancer of the porta hepatis, 43 years after cholangiojejunostomy for congenital biliary dilatation (CBD). This 59-year-old woman had undergone cholangiojejunostomy for CBD at 15 years old. She had remained asymptomatic and no long-term follow-up was performed. However, she developed epigastric pain, and was referred to our hospital after intrahepatic stones were diagnosed.

Imaging showed an irregular stricture of the left hepatic duct and mucosal erythema and irregularity of the opening of the left hepatic duct and cholangiojejunostomy site. Biopsy revealed moderately differentiated adenocarcinoma. Resection of the left hepatic and caudate lobes and site of cholangiojejunostomy was performed. Histopathological examination showed a nodular, well-differentiated adenocarcinoma (T1bN0M0, stage I) in the bile duct.The patient has remained recurrence-free as of 2 years postoperatively. Cholangiojejunostomy for CBD is associated with high risk of cancer, and the prognosis is poor if cancer develops. Patients who have undergone cholangiojejunostomy for CBD may develop complications involving cancer of the residual bile ducts when conditions such as intrahepatic stones develop, and this possibility needs to be addressed through detailed examinations. Some patients, such as the present patient, have not received periodic surveillance after undergoing surgery for CBD during childhood.