-Case Reports-

Mucin-producing Bile Duct Carcinoma Arising from Primary Sclerosing Cholangitis: A Case Report

Shigeki Yokomuro, Yasuo Arima, Yoshiaki Mizuguchi, Tetsuya Shimizu, Yutaka Kawahigashi, Tomohiro Kannda, Masao Arai, Eiji Uchida, Koho Akimaru and Takashi Tajiri

Department of Surgical Regulation of Organ Function and Biology, Nippon Medical School Graduate School of Medicine

A 60-year-old woman with primary sclerosing cholangitis (PSC) and high levels of ALP, γ-GTP, and DUPAN-2 was admitted to our institution for examination. The patient did not have ulcerative colitis or pancreatic intraductal papillary mucinous neoplasm. Imaging studies revealed atypical dilation of bile ducts in the left lobe of the liver. Repeated cytologic examinations of the bile showed atypical cells consistent with adenocarcinoma. The patient underwent extended resection of the left lobe of the liver and was found to have intraductal papillary carcinoma with associated mucin-producing bile duct carcinoma. This carcinoma fills dilated bile duct lumens with mucin. This tumor differs morphologically from typical cholangiocarcinoma, which is usually seen in the late stages of PSC. Just one case of mucin-producing bile duct carcinoma arising from PSC has been reported worldwide. The patient has had no signs of recurrence after 27 months. Patients with mucin-producing bile duct carcinoma, as in the case of its pancreatic counterpart, may have a better prognosis and a higher survival rate than patients with typical cholangiocarcinomas.

J Nippon Med Sch 2007; 74: 61-64

Keywords
mucin-producing bile duct carcinoma, primary sclerosing cholangitis

Correspondence to
Shigeki Yokomuro, Department of Surgical Regulation of Organ Function and Biology, Nippon Medical School Graduate School of Medicine, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan
yokomuro@nms.ac.jp

Received, October 16, 2006
Accepted, November 30, 2006