Toor D, Loree JM, Gao ZH, Wang G, Zhou C. Mixed neuroendocrine-non-neuroendocrine neoplasms of the digestive system: A mini-review. World J Gastroenterol 2022; 28(19): 2076-2087 [PMID: 35664032 DOI: 10.3748/wjg.v28.i19.2076]
Corresponding Author of This Article
Chen Zhou, MD, PhD, Associate Professor, Department of Pathology and Laboratory Medicine, BC Cancer, 600 West 10th Avenue, Vancouver V5Z 1H5, Canada. czhou@bccancer.bc.ca
Research Domain of This Article
Pathology
Article-Type of This Article
Minireviews
Open-Access Policy of This Article
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/
World J Gastroenterol. May 21, 2022; 28(19): 2076-2087 Published online May 21, 2022. doi: 10.3748/wjg.v28.i19.2076
Mixed neuroendocrine-non-neuroendocrine neoplasms of the digestive system: A mini-review
Deepak Toor, Jonathan M Loree, Zu-Hua Gao, Gang Wang, Chen Zhou
Deepak Toor, Zu-Hua Gao, Gang Wang, Chen Zhou, Department of Pathology and Laboratory Medicine, BC Cancer, Vancouver V5Z 1H5, Canada
Deepak Toor, Zu-Hua Gao, Gang Wang, Chen Zhou, Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver V5Z 1H5, Canada
Jonathan M Loree, Department of Medicine, BC Cancer, Vancouver V5Z 4E6, Canada
Author contributions: Toor D performed the majority of the writing; Wang G provided input in writing the paper and contributed histology images; Loree JM provided input and opinions on the clinical management portion of the writing; Gao ZH defined the concepts and ensured the overall quality of the writing; Zhou C designed the outline and coordinated the writing of the paper.
Conflict-of-interest statement: JL has received consulting fees from Taiho, Ipsen, Novartis, Amgen, Eisai, and Bayer; research funding from Ipsen Biopharmaceuticals, Amgen, and Foundation Medicine.
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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Chen Zhou, MD, PhD, Associate Professor, Department of Pathology and Laboratory Medicine, BC Cancer, 600 West 10th Avenue, Vancouver V5Z 1H5, Canada. czhou@bccancer.bc.ca
Received: November 9, 2021 Peer-review started: November 9, 2021 First decision: March 11, 2022 Revised: March 25, 2022 Accepted: April 28, 2022 Article in press: April 28, 2022 Published online: May 21, 2022 Processing time: 189 Days and 8.6 Hours
Abstract
Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) are rare mixed tumors containing both neuroendocrine (NE) and non-NE components. Each component must occupy at least 30% of the tumor volume by definition. Recent molecular evidence suggests MiNENs are clonal neoplasms and potentially harbor targetable mutations similar to conventional carcinomas. There have been multiple changes in the nomenclature and classification of MiNENs which has created some confusion among pathologists on how to integrate the contributions of each component in a MiNEN, an issue which in turn has resulted in confusion in communication with front-line treating oncologists. This mini review summarizes our current understanding of MiNENs and outline diagnosis, prognosis, and management of these neoplasms. The authors emphasize the importance of treating the most aggressive component of the tumor regardless of its percentage volume.
Core Tip: Mixed neuroendocrine neoplasms have been referred to by a long list of names. The latest term, mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN), captures a wider spectrum of neoplasms than did previous nomenclature. This mini review summarizes the development of the term MiNEN and reviews current knowledge about the molecular pathogenesis, diagnosis, prognosis, and management of these neoplasms. MiNENs are viewed as clonal neoplasms and their clinical behaviour and management are ultimately determined by the most aggressive component present.