Retrospective Study
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. May 15, 2023; 15(5): 843-858
Published online May 15, 2023. doi: 10.4251/wjgo.v15.i5.843
Epidemiology and outcome of individuals with intraductal papillary neoplasms of the bile duct
Rong-Shou Wu, Wen-Jun Liao, Jing-Sheng Ma, Jia-Kun Wang, Lin-Quan Wu, Ping Hou
Rong-Shou Wu, Wen-Jun Liao, Jia-Kun Wang, Lin-Quan Wu, Ping Hou, Department of Hepatobiliary and Pancreatic Surgery, The Second Affiliated Hospital of Nanchang University, Nanchang 330000, Jiangxi Province, China
Jing-Sheng Ma, Department of Organ Transplantation, The Second Affiliated Hospital of Nanchang University, Nanchang 330000, Jiangxi Province, China
Ping Hou, Department of General Surgery, The First Affiliated Hospital of Gannan Medical College, No. 23 Qingnian Road, Zhanggong District, Ganzhou 341000, Jiangxi Province, China
Author contributions: Wu RS and Liao WJ contributed equally to this work, Wu RS and Hou P contributed to the conceptualization, formal analysis, software, and writing-original draft; Liao WJ and Wu LQ contributed to the conceptualization, writing-review and editing, project administration; Ma JS and Wang JK contributed to the data curation and methodology. All authors had access to the study data and reviewed and approved the final article. All authors read and approved the final manuscript
Supported by the National Natural Science Foundation of China, No. 81860431 and 82060447; and the Jiangxi Natural Science Foundation, No. 20181BBG70025.
Institutional review board statement: The Surveillance, Epidemiology, and End Results Program of the National Cancer Institute belong to public databases. The patients involved in the database have obtained ethical approval.
Conflict-of-interest statement: The authors declare no conflict of interests.
Data sharing statement: Technical appendix, statistical code, and dataset available from the corresponding author at
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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:
Corresponding author: Ping Hou, PhD, Surgeon, Surgical Oncologist, Department of General Surgery Department, The First Affiliated Hospital of Gannan Medical College, No. 23 Qingnian Road, Zhanggong District, Ganzhou 341000, Jiangxi Province, China.
Received: November 5, 2022
Peer-review started: November 5, 2022
First decision: February 2, 2023
Revised: February 16, 2023
Accepted: March 15, 2023
Article in press: March 15, 2023
Published online: May 15, 2023
Research background

Intraductal papillary neoplasm of the bile duct (IPNB) is a rare distinct subtype of precursor lesions of biliary carcinoma. IPNB is considered to originate from luminal biliary epithelial cells, typically displays mucin-hypersecretion or a papillary growth pattern, and results in cystic dilatation. According to the 2010 World Health Organization classification of tumors in the digestive system, IPNB is defined as the biliary counterpart of intraductal papillary mucinous neoplasm of the pancreas (IPMN) and has identical histopathologic pancreaticobiliary, gastric, intestinal, and oncocytic features. There are still several important differences between IPMN and IPNB exist, unlike invasive IPMN and traditional cholangiocarcinoma in the biliary tract, very little is known about the clinicopathological features and prognostic variables of invasive IPNB.

Research motivation

The epidemiology, tumor characteristics, treatment strategy, and long-term results of invasive IPNB are limited because of the relatively low case numbers.

Research objectives

We conducted a Surveillance, Epidemiology, and End Results (SEER) database evaluation of invasive IPNB to address these shortcomings, and to further elucidate the epidemiological and clinical trends to guide treatment decision-making and to identify further clinical and scientific research areas.

Research methods

Invasive IPNB, IPMN, and traditional cholangiocarcinoma data for affected individuals from 1975 to 2016 were obtained from the SEER database. Annual percentage changes in the incidence and incidence-based (IB) mortality were calculated.

Research results

The incidence and IB mortality of invasive IPNB showed sustained decreases. Similar decreases in incidence and IB mortality were seen for invasive IPMN but not for traditional cholangiocarcinoma. Both overall survival (OS) and cancer-specific survival (CSS) for invasive IPNB were better than for invasive IPMN and traditional cholangiocarcinoma. The most common tumor sites were the pancreaticobiliary ampulla (47.9%) and perihilar tract (36.7%), but the mucin-related subtype of invasive IPNB was the main type, intrahepatically (approximately 90%). In the univariate and multivariate Cox regression analysis, age, tumor site, grade and stage, subtype, surgery, and chemotherapy were associated with OS and CSS (P < 0.05).

Research conclusions

Current population-based study revealed a gradual decrease in the incidence and IB mortality rates of invasive IPNB in the United States population during 1975-2016. The prognosis of invasive IPNB was not only regarding tumor grade and SEER historic stage, but also for different sites and tumor subtypes. Surgery and chemotherapy are associated with improved invasive IPNB outcomes; individuals who do not undergo surgery have the highest risk of death.

Research perspectives

The inspiration of this article is that we found a rare case of hepatic mucoidepidermoid carcinoma (HMEC) in our cancer research center, and found that the malignancy mucinous carcinoma in liver including IPNB, mucinous cystadenocarcinoma and adenosquamous carcinoma. So in our future research direction, we will analyze IPNB cases’ tissues from our center by the next generation sequencing, combined with our published article to analyze the relationship between IPNB and HMEC.