Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Mar 6, 2022; 10(7): 2268-2274
Published online Mar 6, 2022. doi: 10.12998/wjcc.v10.i7.2268
Mixed neuroendocrine-nonneuroendocrine neoplasm of the ampulla: Four case reports
Yang Wang, Zhen Zhang, Cheng Wang, Shi-Hang Xi, Xiao-Ming Wang
Yang Wang, Zhen Zhang, Cheng Wang, Shi-Hang Xi, Xiao-Ming Wang, Department of Hepatobiliary Surgery, The First Affiliated Hospital of Wannan Medical College, Wuhu 241000, Anhui Province, China
Author contributions: Wang Y contribute to data analysis and writing; Zhang Z and Wang C contribute to data collection; Xi SH contribute to manuscript revision; Wang XM contribute to data and manuscript revision; all authors issued final approval for the version to be submitted.
Supported by Funded Project of "Mountain Climbing" Cultivation Project of Yijishan Hospital of Wannan Medical College, No. KPF2019011.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CAREChecklist (2016).
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: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Xiao-Ming Wang, MD, Chief Doctor, Professor, Department of Hepatobiliary Surgery, The First Affiliated Hospital of Wannan Medical College, No. 2 Zheshan West Road, Wuhu 241000, Anhui Province, China. wxm6901@aliyun.com
Received: July 20, 2021
Peer-review started: July 21, 2021
First decision: October 16, 2021
Revised: October 27, 2021
Accepted: January 25, 2022
Article in press: January 25, 2022
Published online: March 6, 2022
Processing time: 224 Days and 11.4 Hours
Abstract
BACKGROUND

Mixed neuroendocrine-nonneuroendocrine neoplasm (MiNEN) is a rare tumor that occurs in the gastrointestinal tract and pancreas, usually composed of adenocarcinoma and neuroendocrine carcinoma. MiNEN occurring in ampulla is even rarer. We report 4 cases of MiNEN in ampulla, combined with literature review to summarize the clinical features and treatment of the disease, in order to improve the understanding of the disease.

CASE SUMMARY

A retrospective analysis was performed in 4 cases of MiNEN of the ampulla diagnosed by pathology from 2014 to 2021. The 4 patients were all male, aged 67-81 years (average 72.25 years). Among them, 2 patients had jaundice, 1 patient had abdominal pain, and 1 patient had jaundice with abdominal pain as the first symptom. All 4 patients underwent enhanced CT or MRI, which all indicated that the tumors were located in the ampulla. Two patients underwent duodenoscopy, and a biopsy revealed ampullary adenocarcinoma. All 4 patients underwent radical pancreaticoduodenectomy. Four cases were followed up: One patient developed severe complications after the operation, his condition deteriorated, and he survived for 1 mo. In the other 3 patients, tumor recurrence was observed during follow-up, and 2 of them survived for 29 mo and 22 mo respectively. One case survived and is still being followed up.

CONCLUSION

MiNEN of the ampulla are extremely rare, lacking typical clinical symptoms and imaging features, and are usually diagnosed after postoperative histopathological and immunohistochemical examinations. The main treatment is radical surgical resection, which can be combined with chemotherapy. The best method of diagnosis and treatment needs further research.

Keywords: Ampulla; Mixed neuroendocrine-nonneuroendocrine neoplasm; Pancreaticoduodenectomy; Chemotherapy; Case report

Core Tip: Mixed neuroendocrine-nonneuroendocrine neoplasm (MiNEN) is a rare tumor that occurs in the gastrointestinal tract and pancreas. MiNEN occurring in ampulla is extremely rare. We report 4 cases of MiNEN of the ampulla, summarizes these cases and reviews related literature.