title: Cholangiocarcinoma developed in a patient with IgG4-related disease.
abstract: A 77-year-old man with jaundice and a pancreatic head tumor was referred to our hospital in August 2006. The initial laboratory tests, computed tomography (CT) scan, magnetic resonance imaging (MRI), and endoscopic retrograde cholangiopancreatography suggested IgG4-related cholangitis and autoimmune pancreatitis. Oral prednisolone (PSL) was then administered. This treatment reduced the size of the pancreatic parenchyma, and the lower common bile duct (CBD) returned to its normal size. Thus, the oral PSL was gradually tapered to a maintenance dose. In February 2010, a CT scan and MRI showed segmental wall thickening and stenosis of the middle CBD, the progression of which led to extrahepatic obstructive jaundice. We suspected the emergence of a cholangiocarcinoma rather than the exacerbation of the IgG4-related sclerosing cholangitis because the stricture of the CBD was short and localized. Then, a percutaneous transhepatic biliary drainage was performed. The biopsy specimens obtained via the percutaneous transhepatic tract indicated an abnormal glandular formation, suggesting the presence of a moderate, well-differencated adenocarcinoma. The gross examination, microscopic examination and immunohistochemical analysis of the pancreaticoduodenectomy specimen suggested that a cholangiocarcinoma developed from the IgG4-related sclerosing cholangitis.
authors.affiliation: Akitoshi Douhara, Akira Mitoro, Emi Otani, Masanori Furukawa, Kosuke Kaji, Masakazu Uejima, Masayoshi Sawai, Motoyuki Yoshida, Hitoshi Yoshiji, Junichi Yamao, Hiroshi Fukui, Department of Gastroenterology and Endocrinology, Nara Medical University, Nara 634-8521, Japan.
authors.forename: Akitoshi
authors.initials: A
authors.lastname: Douhara
authors.forename: Akira
authors.initials: A
authors.lastname: Mitoro
authors.forename: Emi
authors.initials: E
authors.lastname: Otani
authors.forename: Masanori
authors.initials: M
authors.lastname: Furukawa
authors.forename: Kosuke
authors.initials: K
authors.lastname: Kaji
authors.forename: Masakazu
authors.initials: M
authors.lastname: Uejima
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Sawai
authors.forename: Motoyuki
authors.initials: M
authors.lastname: Yoshida
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Yoshiji
authors.forename: Junichi
authors.initials: J
authors.lastname: Yamao
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Fukui
keywords: Autoimmune pancreatitis; Cholangiocarcinoma; IgG4; Sclerosing cholangitis