Review
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Surg. Mar 27, 2021; 13(3): 231-255
Published online Mar 27, 2021. doi: 10.4240/wjgs.v13.i3.231
Gastroenteropancreatic neuroendocrine neoplasms: A clinical snapshot
Cornelius J Fernandez, Mayuri Agarwal, Biju Pottakkat, Nisha Nigil Haroon, Annu Susan George, Joseph M Pappachan
Cornelius J Fernandez, Mayuri Agarwal, Department of Endocrinology and Metabolism, Pilgrim Hospital, United Lincolnshire Hospitals NHS Trust, Boston PE21 9QS, United Kingdom
Biju Pottakkat, Department of Surgical Gastroenterology, Jawaharlal Institute of Post Graduate Medical Education and Research (JIPMER), Puducherry 605006, India
Nisha Nigil Haroon, Department of Endocrinology and Internal Medicine, Northern Ontario School of Medicine, Sudbury P3E 2C6, Ontario, Canada
Annu Susan George, Department of Medical Oncology, VPS Lakeshore Hospital, Cochin 682040, Kerala, India
Joseph M Pappachan, Department of Endocrinology and Metabolism, Lancashire Teaching Hospitals NHS Trust, PR2 9HT, Preston, The University of Manchester, Oxford Road M13 9PL, Manchester Metropolitan University, All Saints Building M15 6BH, Manchester, United Kingdom.
Author contributions: Fernandez CJ and Agarwal M performed majority of the initial drafting and prepared the figures and tables, and share the first authorship; Pottakkat B made critical revisions in the surgical aspects of the write up; Haroon NN provided substantial additional contributions to the endocrine aspects of the work; George AS contributed to the conception and design of the study and made critical revisions especially the oncological aspects; Pappachan JM conceived the study, made critical revisions and provided final approval of the final version of the manuscript to be published
Conflict-of-interest statement: Authors declare no conflict of interests for this article.
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: Joseph M Pappachan, MD, MRCP, FRCP; Consultant, Senior Researcher, Department of Endocrinology and Metabolism, Lancashire Teaching Hospitals NHS Trust, PR2 9HT, Preston, The University of Manchester, Oxford Road M13 9PL, Manchester Metropolitan University, All Saints Building M15 6BH, Manchester, United Kingdom. drpappachan@yahoo.co.in
Received: December 11, 2020
Peer-review started: December 11, 2020
First decision: January 11, 2021
Revised: January 17, 2021
Accepted: March 2, 2021
Article in press: March 2, 2021
Published online: March 27, 2021
Processing time: 96 Days and 18.2 Hours
Core Tip

Core Tip: Gut, the largest endocrine organ of the body, secretes several hormones through its specialised neuroendocrine cells. Neuroendocrine neoplasms (NENs) originating from these cells are collectively known as gastroenteropancreatic (GEP) NENs that pose significant challenges to clinicians in the diagnostic work up, prognostication and management. Rapid advancements in the molecular diagnostic and therapeutic strategies in the recent years revolutionised the options for clinical investigations and management of patients with these uncommon neoplasms. This review updates the pathophysiological aspects, diagnostic strategies, and management algorithms of GEP NENs with the best evidence currently available in the global scientific literature to enable clinicians to optimally manage these unusual disease entities.