Combined adult-to-adult living donor right lobe liver transplantation and pancreatoduodenectomy for distal bile duct adenocarcinoma in a patient with primary sclerosing cholangitis.

Abstract

Liver transplantation is the best therapeutic option for patients with end-stage liver disease from primary sclerosing cholangitis. Primary sclerosing cholangitis is associated with a markedly increased risk of cholangiocarcinoma, which adversely affects survival. Approximately 20% to 30% of cholangiocarcinomas are localized in the distal bile duct. Pancreatoduodenectomy is the curative therapy for cholangiocarcinomas in this location. We reviewed our data on a patient with primary sclerosing cholangitis-related end-stage liver disease and a simultaneous distal bile duct tumor, which was treated with a combined right-lobe, living-donor liver transplantation and pancreatoduodenectomy. The patient was discharged 32 days post-transplantation. He is currently alive 1 year after the procedure with no evidence of recurrent cancer. Combined living-donor liver transplantation and pancreatoduodenectomy is feasible and allows timely and elective surgical control of carefully selected distal bile duct tumors in the setting of end-stage liver disease.