Korean J Pancreas Biliary Tract.  2016 Jul;21(3):168-173. 10.15279/kpba.2016.21.3.168.

Cholangiocarcinoma Masquerading as IgG4-related Sclerosing Cholangitis

  • 1Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. mhkim@amc.seoul.kr
  • 2Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.


IgG4-related sclerosing cholangitis (IgG4-SC) represents a rare but clinically challenging differential diagnosis in patients with biliary strictures which can be mistaken for cholangiocarcinoma. We present a case of a 73-year-old male presented with abdominal discomfort and weight loss. Biliary images showed long-segment luminal narrowing of extrahepatic bile duct associated with prominent enhanced wall thickening, but luminal patency was preserved. Pancreatic images revealed segmental irregular narrowing of main pancreatic duct without upstream duct dilatation. His liver function tests and CA19-9 level were normal. Putting all findings together, IgG4-SC associated with autoimmune pancreatitis was strongly suspected. However, endobiliary biopsy of extrahepatic bile duct revealed adenocarcinoma which was not resectable due to celiac axis involvement. Because there is an overlap in biliary imaging findings between IgG4-SC and cholangiocarcinoma, biopsy is essential for adequate differential diagnosis. We present a case of cholangiocarcinoma masquerading as IgG4-SC based on clinical and imaging findings.


IgG4-related sclerosing cholangitis; Cholangiocarcinoma; Autoimmune pancreatitis
Full Text Links
export Copy
  • Twitter
  • Facebook
Similar articles
Copyright © 2021 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr