Cholangiocarcinoma Resembling IgG4-related Sclerosing Cholangitis

Kazuaki Akahoshi; Atsushi Kanno; Tetsurou Miwata; Hiroki Nagai; Kensuke Yokoyama; Eriko Ikeda; Kozue Ando; Kiichi Tamada; Noriyoshi Fukushima; Alan Kawarai Lefor; Hironori Yamamoto

doi:10.2169/internalmedicine.1144-22

This article has now been updated. Please use the final version.

Cholangiocarcinoma Resembling IgG4-related Sclerosing Cholangitis

Kazuaki Akahoshi, Atsushi Kanno, Tetsurou Miwata, Hiroki Nagai, Kensuke Yokoyama, Eriko Ikeda, Kozue Ando, Kiichi Tamada, Noriyoshi Fukushima, Alan Kawarai Lefor, Hironori Yamamoto

Author information

Keywords: Immunoglobulin G4-related sclerosing cholangitis, cholangiocarcinoma, immunoglobulin G4-related disease, Sister Mary Joseph's nodule

JOURNAL OPEN ACCESS Advance online publication

Article ID: 1144-22

DOI https://doi.org/10.2169/internalmedicine.1144-22

The final version of this article is now available: Vol. 62 (2023), No. 23 pp. 3495-3500

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Abstract

A 66-year-old man diagnosed with immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) with diffuse intrahepatic bile duct stenosis and elevated serum IgG4 levels was referred for a further examination because of elevated serum CA19-9 levels despite treatment with corticosteroids. An umbilical nodule was found on a physical examination and a biopsy showed adenocarcinoma. Although several imaging studies revealed no changes from prior studies, bile cytology collected by endoscopic retrograde cholangiopancreatography showed adenocarcinoma. Consequently, the patient was diagnosed with cholangiocarcinoma resembling IgG4-SC after detecting an umbilical metastasis, also known as Sister Mary Joseph's nodule.

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