Cholangiocarcinoma with histological findings of IgG4-related sclerosing cholangitis

Abstract

A 79-year-old man presented with abdominal pain. It showed a rise of the tumor marker CA19-9 (182U/ml) in the blood test. The computed tomography (CT) scan revealed thickening of the bile duct wall. The endoscopic retrograde cholangiopancreatography (ERCP) showed narrowing of the lower common bile duct. An ill-defined lesion was confirmed in extrahepatic bile duct. As transpapillary bile duct biopsy showed moderately differentiated adenocarcinoma. The positron emission tomography (PET) showed integrated and MaxSUV 3.6. We diagnosed extrahepatic cholangiocellular carcinoma. Subtotal Stomach-Preserving Pancreaticoduodenectomy were performed. Pathological examinations showed the extrahepatic cholangiocellular carcinoma with Lymph node metastasis (UICC, pT2 N1, M0 Stage IIB). Microscopically, Immunohistochemistry was performed to identify IgG4-positive plasma cells and the storiform fibrosis. More than 10 IgG4-positive plasma cells/high power fields were detected in the CBD. The bile duct wall showed the typical features of IgG4-related sclerosing cholangitis. This case was cholangiocarcinoma accompanied by fibroinflammatory lesions with IgG4-positive plasma cells. Proactive pathological examination is needed to detect potential cholangiocarcinomas.