Case Report
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World J Gastroenterol. Nov 14, 2014; 20(42): 15925-15930
Published online Nov 14, 2014. doi: 10.3748/wjg.v20.i42.15925
Intraductal papillary neoplasm of the bile duct developing in a patient with primary sclerosing cholangitis: A case report
Hiroyuki Hachiya, Junji Kita, Takayuki Shiraki, Yukihiro Iso, Mitsugi Shimoda, Keiichi Kubota
Hiroyuki Hachiya, Junji Kita, Takayuki Shiraki, Yukihiro Iso, Mitsugi Shimoda, Keiichi Kubota, Second Department of Surgery, Dokkyo Medical University, Tochigi 321-0293, Japan
Author contributions: Hachiya H designed the report; Hachiya H, Kita J, Shiraki T, Iso Y and Shimoda M were attending doctors for the patient; Kubota K performed the surgery; Hachiya H organized the report and wrote the paper; Kubota K and Kita J revised the paper for intellectual content.
Correspondence to: Hiroyuki Hachiya, MD, Second Department of Surgery, Dokkyo Medical University, 880 Kita Kobayashi, Mibu, Tochigi 321-0293, Japan. hachiya8@dokkyomed.ac.jp
Telephone: +81-282-861111 Fax: +81-282-866317
Received: March 18, 2014
Revised: May 4, 2014
Accepted: June 26, 2014
Published online: November 14, 2014
Abstract

We report a case of intraductal papillary neoplasm of the bile duct (IPNB) that developed in a patient with primary sclerosing cholangitis. A 46-year-old woman was admitted to our hospital with obstructive jaundice. The liver function tests demonstrated increased serum liver enzyme levels. Computed tomography showed dilatation of the intrahepatic bile ducts. Abdominal ultrasonography revealed a highly echoic protruding lesion in the posterior bile duct near the right lobe of the liver. The lesion was suspected to be IPNB, but we were unable to confirm whether it was a carcinoma. A right hepatectomy was performed, and this showed that the dilated bile duct was filled with mucin and contained several yellowish papillary tumors. Histologically, the neoplastic biliary epithelium showed papillary growth in the dilated lumen. The tumor was diagnosed as IPNB, high-grade intraepithelial neoplasia secreting abundant mucin. No recurrence has been detected 3 years after surgery.

Keywords: Intraductal papillary neoplasm of the bile duct, Primary sclerosing cholangitis, Cholangiocarcinoma, High-grade intraepithelial neoplasia, Premalignant lesion

Core tip: Intraductal papillary neoplasm of the bile duct (IPNB) is a variant of bile duct carcinoma that is characterized by grossly visible lesions and better outcomes compared with cholangiocarcinoma (CCA). Primary sclerosing cholangitis (PSC) is considered to be a risk factor for CCA. We report a 46-year-old woman who presented with dilatation of the intrahepatic bile ducts and mucin in the common bile duct. This is the first case of PSC in which IPNB developed in the setting of high-grade intraepithelial neoplasia.