Segmental cholangiectasia clinically worrisome for cholangiocarcinoma: comparison with recurrent pyogenic cholangitis

The aim of this study was to review the clinical, radiographic, and pathologic features of cases of benign segmental cholangiectasia in non-Asian US patients with clinical concern for cholangiocarcinoma and compare these features with cases of recurrent pyogenic cholangitis (RPC) in Asian patients. A total of 10 non-Asian US patients with benign segmental cholangiectasia were included in this study. Nine of them underwent partial hepatic resection due to cholangiographic findings of segmental cholangiectasia with mural thickening and/or proximal biliary stricture. One was found to have markedly dilated and thickened intrahepatic bile ducts at the time of autopsy. Clinical and radiographic findings were reviewed. Elastin stains and immunostains for immunoglobulin G4, cluster of differentiation (CD1a), and Langerin were performed. Six comparison cases of RPC in Asian US patients were also examined. Histologic examination of resection specimens revealed markedly dilated large intrahepatic bile ducts with variable degrees of mural fibrosis, periductal gland hyperplasia, inflammation, and liver parenchymal atrophy. These changes were not associated with a ductular reaction. There was no evidence of biliary dysplasia or biliary cirrhosis in any cases. No gross or microscopic feature definitively separated the Asian from non-Asian patients. The etiology of this disorder in non-Asian US patients is unclear. It does not appear to represent a localized variant of Caroli disease or primary sclerosing cholangitis. The high degree of similarity shared by these cases and classic RPC suggests a common pathogenic mechanism, although the pathologic features tend to be less well developed in the cases from the non-Asian US patients.