2019 Volume 52 Issue 1 Pages 36-44
A 79-year-old woman was found to have an elevated serum bilirubin level by her family doctor and was referred to our hospital. Enhanced abdominal CT showed wall thickening of the hilar bile duct in a wide area from the proximal common hepatic duct to the right anterior branch, right posterior branch, and left hepatic ducts, resulting in diffuse biliary stricture and dilation of both intrahepatic ducts. The right hepatic artery and A4 were in contact with the thickened biliary wall, suggesting tumor involvement of these arteries. ERCP demonstrated an irregular stricture of the hilar bile duct, which was consistent with the CT findings. The patient underwent biliary drainage from B2. Although cytology of the bile revealed no malignancy, we diagnosed hilar cholangiocarcinoma based on the imaging findings. We conducted right hepatic trisegmentectomy and caudate lobectomy with extrahepatic bile duct resection and lymphadenectomy. Gross appearance of the cut surface of the specimen revealed marked wall thickening in the bile duct of the hepatic hilum. On histological and immunohistochemical examination, the bile duct was lined by normal biliary epithelium and thickened by marked formation of lymphoid follicles with germinal centers and proliferation of fibrous stroma. Only a small amount of IgG4-positive plasma cells was seen. These findings were consistent with follicular cholangitis. She is doing well without recurrence of sclerosing cholangitis for over 4.5 years after the surgery. Although a review of the literature revealed this to be a rare case, follicular cholangitis should be considered as a differential diagnosis of biliary stricture of the hepatic hilum.
