Case Report
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World J Gastrointest Oncol. Aug 15, 2013; 5(8): 181-185
Published online Aug 15, 2013. doi: 10.4251/wjgo.v5.i8.181
Cholangiocarcinoma developed in a patient with IgG4-related disease
Akitoshi Douhara, Akira Mitoro, Emi Otani, Masanori Furukawa, Kosuke Kaji, Masakazu Uejima, Masayoshi Sawai, Motoyuki Yoshida, Hitoshi Yoshiji, Junichi Yamao, Hiroshi Fukui
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
Author contributions: Douhara A and Mitoro A designed the report; Mitoro A was the attending doctor for the patient; Otani E, Furukawa M, Kaji K, Uejima M and Yoshiji H discussed the pathogenesis; Douhara A, Mitoro A, Sawai M, Yoshida M and Yamao J performed endoscopic examinations; Fukui H organized the report; and Douhara A wrote the paper.
Correspondence to: Akitoshi Douhara, MD, Department of Gastroenterology and Endocrinology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara 634-8521, Japan. aki-do@hotmail.co.jp
Telephone: +81-744-223051 Fax: +81-744-224121
Received: June 19, 2013
Revised: August 5, 2013
Accepted: August 11, 2013
Published online: August 15, 2013
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.

Keywords: IgG4, Sclerosing cholangitis, Autoimmune pancreatitis, Cholangiocarcinoma

Core tip: Chronic biliary inflammation and cholestasis are risk factors for cholangiocarcinoma. However, an association of IgG4-related sclerosing cholangitis with cholangiocarcinoma has not been previously demonstrated. To date, only two report described have neoplasia in the bile duct in patients with IgG4-related sclerosing cholangitis. Risk factors for cholangiocarcinoma include chronic biliary inflammation and cholestasis, both of which can contribute to the malignant transformation of cholangiocytes in patients with sclerosing cholangitis. Because of a long duration of overt IgG4-related sclerosing cholangitis and the affected location of the patient’s cholangiocarcinoma, we presumed a cause-and-effect relationship between the sclerosing cholangitis and bile duct cancer.