Case Report
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Feb 21, 2016; 22(7): 2391-2397
Published online Feb 21, 2016. doi: 10.3748/wjg.v22.i7.2391
Cystic micropapillary neoplasm of peribiliary glands with concomitant perihilar cholangiocarcinoma
Tsuneyuki Uchida, Yusuke Yamamoto, Takaaki Ito, Yukiyasu Okamura, Teiichi Sugiura, Katsuhiko Uesaka, Yasuni Nakanuma
Tsuneyuki Uchida, Yusuke Yamamoto, Takaaki Ito, Yukiyasu Okamura, Teiichi Sugiura, Katsuhiko Uesaka, Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center Hospital, Sunto-gun, Shizuoka 4118777, Japan
Tsuneyuki Uchida, Yasuni Nakanuma, Division of Diagnostic Pathology, Shizuoka Cancer Center Hospital, Sunto-gun, Shizuoka 4118777, Japan
Author contributions: Uchida T, Uesaka K and Nakanuma Y did study design and manuscript preparation; Uchida T, Yamamoto Y, Ito T, Okamura Y, Sugiura T and Uesaka K did clinical diagnosis and management; Nakanuma Y contributed to pathological diagnosis.
Institutional review board statement: The study was reviewed and approved by the Shizuoka Cancer Center Hospital Institutional review board.
Conflict-of-interest statement: None of the authors has a conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Yasuni Nakanuma, MD, Division of Diagnostic Pathology, Shizuoka Cancer Center Hospital, 1007 Shimonagakubo, Nagaizumi-cho, Sunto-gun, Shizuoka 4118777, Japan. y.nakanuma@scchr.jp
Telephone: +81-55-9895222 Fax: +81-55-9895783
Received: August 3, 2015
Peer-review started: August 5, 2015
First decision: September 29, 2015
Revised: October 23, 2015
Accepted: November 24, 2015
Article in press: November 24, 2015
Published online: February 21, 2016
Processing time: 180 Days and 13.7 Hours
Abstract

We report a case of a 75-year-old man with cystic micropapillary neoplasm of peribiliary glands detected preoperatively by radiologic examination. Enhanced computed tomography showed a low-density mass 2.2 cm in diameter in the right hepatic hilum and a cystic lesion around the common hepatic duct. Under a diagnosis of perihilar cholangiocarcinoma, right hepatectomy with caudate lobectomy and bile duct resection were performed. Pathological examination revealed perihilar cholangiocarcinoma mainly involving the right hepatic duct. The cystic lesion was multilocular and covered by columnar lining epithelia exhibiting increased proliferative activity and p53 nuclear expression; it also contained foci of micropapillary and glandular proliferation. Therefore, the lesion was diagnosed as a cystic micropapillary neoplasm of peribiliary glands and resembled flat branch-type intraductal papillary mucinous neoplasm of the pancreas. Histological examination showed the lesion was discontinuous with the perihilar cholangiocarcinoma. Immunohistochemistry showed the cystic neoplasm was strongly positive for MUC6 and that the cholangiocarcinoma was strongly positive for MUC5AC and S100P. These results suggest these two lesions have different origins. This case warrants further study on whether this type of neoplasm is associated with concomitant cholangiocarcinoma as observed in pancreatic intraductal papillary mucinous neoplasm with concomitant pancreatic duct adenocarcinoma.

Keywords: Intraductal papillary neoplasm of the bile duct; Peribiliary gland; Gastric type; Perihilar cholangiocarcinoma

Core tip: This report highlights intraductal papillary neoplasm of bile duct (IPNB) arising from peribiliary glands (PBGs) in a case of perihilar cholangiocarcinoma, which is of special interest because to our knowledge this is the first report of IPNB arising from PBGs with concomitant perihilar cholangiocarcinoma occurred separately, and the neoplasm may be regarded as the biliary counterpart of branch duct-type intraductal papillary mucinous neoplasm of pancreas with concomitant pancreatic duct adenocarcinoma.