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Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Sep 21, 2021; 27(35): 5890-5907
Published online Sep 21, 2021. doi: 10.3748/wjg.v27.i35.5890
Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies
Roberta Elisa Rossi, Alessandra Elvevi, Davide Citterio, Jorgelina Coppa, Pietro Invernizzi, Vincenzo Mazzaferro, Sara Massironi
Roberta Elisa Rossi, Davide Citterio, Jorgelina Coppa, Vincenzo Mazzaferro, HPB Surgery, Hepatology and Liver Transplantation, ENETS Center of Excellence, Fondazione IRCCS Istituto Nazionale Tumori (INT, National Cancer Institute), Milan 20133, Italy
Roberta Elisa Rossi, Department of Pathophysiology and Transplantation, University of Milan, Milan 20122, Italy
Alessandra Elvevi, Pietro Invernizzi, Sara Massironi, Division of Gastroenterology and Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, University of Milano-Bicocca, Monza 20900, Italy
Alessandra Elvevi, Pietro Invernizzi, Sara Massironi, European Reference Network on Hepatological Diseases (ERN RARE-LIVER), San Gerardo Hospital, Monza 20033, Italy
Vincenzo Mazzaferro, Department of Oncology and Hemato-Oncology, University of Milan, Milan 20122, Italy
Author contributions: Rossi RE designed the research; Rossi RE, Elvevi A, Citterio D, and Massironi S performed the literature search and wrote the first draft of the paper; Rossi RE, Massironi S, Coppa J, Invernizzi P, and Mazzaferro V revised the manuscript for important intellectual content; Rossi RE and Massironi S wrote the final version of the paper; All of the authors approved the final version for publication.
Conflict-of-interest statement: The authors declare no conflict of interest 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: Roberta Elisa Rossi, MD, PhD, HPB Surgery, Hepatology and Liver Transplantation, ENETS Center of Excellence, Fondazione IRCCS Istituto Nazionale Tumori (INT, National Cancer Institute), via Giacomo Venezian, Milan 20133, Italy. robertaelisa.rossi@gmail.com
Received: March 15, 2021
Peer-review started: March 15, 2021
First decision: April 17, 2021
Revised: April 29, 2021
Accepted: August 10, 2021
Article in press: August 10, 2021
Published online: September 21, 2021
Core Tip

Core Tip: As symptoms of Zollinger-Ellison syndrome are nonspecific and overlap with other gastrointestinal disorders, most of these patients are usually referred to general gastroenterologists, leading to a diagnostic delay. A better disease awareness together with the maintenance of a high index of suspicion are necessary to make the final diagnosis. The proper management of Zollinger-Ellison syndrome due to a gastrinoma include both the medical treatment for symptom’s relief and surgery whenever feasible with curative intent; the multidisciplinary approach, with close cooperation between gastroenterologists and surgeons, and the referral to tertiary centers with great expertise in the neuroendocrine field are mandatory.