IgG4-related Sclerosing Cholangitis Complicated with Cholangiocarcinoma and Detected by Forkhead Box P3 Immunohistochemical Staining

• Published in: Internal Medicine Vol. 60; no. 6; pp. 859 - 866
• Main Authors: Toyohara, Tadashi, Nakazawa, Takahiro, Zakharia, Kais, Shimizu, Shuya, Miyabe, Katsuyuki, Harada, Kenichi, Notohara, Kenji, Yamada, Tomohiro, Hayashi, Katsumi, Naitoh, Itaru, Hayashi, Kazuki, Kataoka, Hiromi
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 15.03.2021; Japan Science and Technology Agency
• Subjects: Adenocarcinoma; Aged, 80 and over; Autoimmune Diseases - diagnosis; autoimmune pancreatitis; Bile; Bile Duct Neoplasms - complications; Bile Duct Neoplasms - diagnosis; Bile ducts; Bile Ducts, Intrahepatic; Case Report; Cholangiocarcinoma; Cholangiocarcinoma - complications; Cholangiocarcinoma - diagnosis; Cholangitis; Cholangitis, Sclerosing - complications; Cholangitis, Sclerosing - diagnosis; Diagnosis, Differential; forkhead box P3; Forkhead protein; Foxp3 protein; Humans; Immunoglobulin G; Immunoglobulin G4; immunoglobulin G4-related disease; immunoglobulin G4-related sclerosing cholangitis; Internal medicine; Male; N-Terminus; Pancreatitis; Staining and Labeling; Stricture; immunoglobulin G4-related disease; immunoglobulin G4-related sclerosing cholangitis; autoimmune pancreatitis; cholangiocarcinoma; forkhead box P3
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.5920-20

An 80-year-old man was admitted due to biliary stricture with autoimmune pancreatitis. Although radiographical examinations suggested Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC), punched biopsies from the bile duct revealed adenocarcinoma. In the resected specimen, abundant N-terminus of Forkhead box P3 (Foxp3)-positive cells were localized in cholangiocarcinoma (CCA) tissue, while IgG4-positive cells were spread around the entire bile duct. Therefore, the case was diagnosed with IgG4-SC accompanied by CCA, not sporadic CCA. We herein report an informative case wherein IgG4-positive cells were abundant in CCA tissue and Foxp3 immunohistochemical staining allowed us to determine that this case had two entities.