Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Feb 21, 2015; 21(7): 2254-2259
Published online Feb 21, 2015. doi: 10.3748/wjg.v21.i7.2254
Mixed adenoneuroendocrine carcinoma of the ampulla: Two case reports
Zhen Huang, Wei-Dong Xiao, Yong Li, Song Huang, Jun Cai, Jian Ao
Zhen Huang, Wei-Dong Xiao, Yong Li, Jun Cai, Jian Ao, Department of General Surgery, the First Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi Province, China
Song Huang, Department of Pathology, the First Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi Province, China
Author contributions: Huang Z collected the clinical data and wrote the paper; Li Y, Xiao WD and Cai J performed the operation; Huang S made the pathologic diagnosis; Ao J assisted in the reference search.
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: Wei-Dong Xiao, MD, PhD, Department of General Surgery, the First Affiliated Hospital of Nanchang University, No.17, Yongwai Zhengjie, Nanchang 330006, Jiangxi Province, China. frankxwd@126.com
Telephone: +86-791-88694131 Fax: +86-791-88694131
Received: August 2, 2014
Peer-review started: August 4, 2014
First decision: August 27, 2014
Revised: September 22, 2014
Accepted: November 7, 2014
Article in press: November 11, 2014
Published online: February 21, 2015
Processing time: 192 Days and 20.3 Hours
Abstract

Mixed adenoneuroendocrine carcinoma (MANEC) is a malignant tumor with adenocarcinoma and neuroendocrine components, with ≥ 30% of each component required. MANEC of the ampulla is rare. To the best of our knowledge, only 15 cases of MANEC of the ampulla have been reported in the English-language literature. Here, we report two cases of MANEC of the ampulla in two women aged 43 and 60 years, which was confirmed by histology after pancreaticoduodenectomy. These tumors contained neuroendocrine and adenocarcinoma components. The neuroendocrine components were positive for chromogranin A (CgA), synaptophysin (Syn) and CD56 by immunostaining. The adenocarcinoma components were negative for CgA, Syn and CD56. Both cases were T3N0M0 (Stage IIIA). They survived for 15 and 20 mo after surgery, respectively. A brief discussion about the histopathological features, clinical behavior and treatment of MANEC of ampulla, and review of the relevant literature are presented.

Keywords: Neuroendocrine tumor; Mixed adenoneuroendocrine carcinoma; Composite carcinoma; Ampulla; Immunohistochemical

Core tip: The authors report two cases of mixed adenoneuroendocrine carcinoma (MANEC) of the ampulla, which is an extremely rare disease. Both patients received pancreaticoduodenectomy and chemotherapy after surgery. The cases provide a brief discussion about the histopathological features, clinical behavior and treatment of MANEC of the ampulla based on previously published studies.