Retrospective Study
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Apr 14, 2015; 21(14): 4261-4267
Published online Apr 14, 2015. doi: 10.3748/wjg.v21.i14.4261
Biliary tract intraductal papillary mucinous neoplasm: Report of 19 cases
Xing Wang, Yun-Qiang Cai, Yong-Hua Chen, Xu-Bao Liu
Xing Wang, Yun-Qiang Cai, Yong-Hua Chen, Xu-Bao Liu, Department of Hepatopancreatobiliary Surgery, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
Author contributions: Wang X and Liu XB designed the research; Wang X, Cai YQ, and Chen YH performed the research and analyzed the data; Wang X and Cai YQ wrote the paper; Liu XB proofread and revised the manuscript; all authors approved the version to be published.
Supported by The Research Special Fund for Public Welfare Industry of Health, No. 201202007.
Conflict-of-interest: There is no conflict of interest of the authors.
Data sharing: No additional data are available.
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: Xu-Bao Liu, MD, PhD, Department of Hepatopancreatobiliary Surgery, West China Hospital, Sichuan University, No. 37 GuoXue Alley, Chengdu 610041, Sichuan Province, China. shoubulele@gmail.com
Telephone: +86-28-85422477 Fax: +86-28-85422474
Received: September 2, 2014
Peer-review started: September 3, 2014
First decision: November 14, 2014
Revised: November 21, 2014
Accepted: January 8, 2015
Article in press: January 8, 2015
Published online: April 14, 2015
Processing time: 224 Days and 13.8 Hours
Abstract

AIM: To gain a better understanding of biliary tract intraductal papillary mucinous neoplasm (BT-IPMN).

METHODS: From January 2000 to December 2013, 19 cases of BT-IPMN were retrospectively identified from a total of 343 biliary tract tumors resected in our single institution. Demographic characteristics, clinical data, pathology, surgical strategies, and long-term follow-up were analyzed.

RESULTS: The mean age of the 19 BT-IPMN cases was 53.8 years (range: 25-74 years). The most common symptom was abdominal pain (15/19; 78.9%), followed by jaundice (7/19; 36.8%). Cholangitis was associated with most (16/19; 84.2%) of the BT-IPMN cases. Macroscopically visible mucin was detected in all 19 patients, based on original surgical reports. The most common abnormal preoperative imaging findings for BT-IPMN were bile duct dilation (19/19; 100%) and intraluminal masses (10/19; 52.6%). Thirteen (68.4%) cases involved the intrahepatic bile duct and hilum. We performed left hepatectomy in 11/19 (57.9%), right hepatectomy in 2/19 (10.5%), bile duct resection in 4/19 (21.1%), and pancreatoduodenectomy in 1/19 (5.3%) patients. One (5.3%) patient was biopsied and received a choledochojejunostomy because of multiple tumors involving the right extrahepatic and left intrahepatic bile ducts. Histology showed malignancy in 10/19 (52.6%) patients. The overall median survival was 68 mo. The benign cases showed a non-significant trend towards improved survival compared to malignant cases (68 mo vs 48 mo, P = 0.347). The patient without tumor resection died of liver failure 22 mo after palliative surgery.

CONCLUSION: BT-IPMN is a rare biliary entity. Complete resection of the tumor is associated with good survival, even in patients with malignant disease.

Keywords: Biliary tract; Cystic tumor; Intraductal papillary mucinous neoplasm; Mucinous tumor; Papillary tumor

Core tip: Our study involved a large number of patients with biliary tract intraductal papillary mucinous neoplasm (BT-IPMN) from a large Chinese institution. We summarized the clinical features, radiologic findings, pathology, surgical strategies, and long-term follow-up of these patients to achieve a better understanding of this rare disease. Our findings indicated that BT-IPMN is a rare biliary entity and complete resection of the tumor is associated with good survival, even in patients with malignant disease.