Dai M, Mullins CS, Lu L, Alsfasser G, Linnebacher M. Recent advances in diagnosis and treatment of gastroenteropancreatic neuroendocrine neoplasms. World J Gastrointest Surg 2022; 14(5): 383-396 [PMID: 35734622 DOI: 10.4240/wjgs.v14.i5.383]
Corresponding Author of This Article
Michael Linnebacher, PhD, Academic Fellow, Research Fellow, Research Scientist, Senior Researcher, Senior Scientist, Clinic of General Surgery, Molecular Oncology and Immunotherapy, Rostock University Medical Center, Schillingallee 35, Rostock 18057, Mecklenburg-Vorpommern, Germany. michael.linnebacher@med.uni-rostock.de
Research Domain of This Article
Oncology
Article-Type of This Article
Review
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 Gastrointest Surg. May 27, 2022; 14(5): 383-396 Published online May 27, 2022. doi: 10.4240/wjgs.v14.i5.383
Recent advances in diagnosis and treatment of gastroenteropancreatic neuroendocrine neoplasms
Meng Dai, Christina S Mullins, Lili Lu, Guido Alsfasser, Michael Linnebacher
Meng Dai, Christina S Mullins, Lili Lu, Michael Linnebacher, Clinic of General Surgery, Molecular Oncology and Immunotherapy, Rostock University Medical Center, 18057 Rostock, Germany
Guido Alsfasser, Clinic of General Surgery, Rostock University Medical Center, 18057 Rostock, Germany
Author contributions: Dai M and Linnebacher M analyzed the literature and wrote the manuscript; Alsfasser G, Lu L and Mullins CS critically reviewed the manuscript; Mullins CS edited the language as a native speaker.
Supported byChina Scholarship Council, No. 202108080085 to (Dai M) and No. 201908080127 to (Lu LL).
Conflict-of-interest statement: None to be declared.
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: Michael Linnebacher, PhD, Academic Fellow, Research Fellow, Research Scientist, Senior Researcher, Senior Scientist, Clinic of General Surgery, Molecular Oncology and Immunotherapy, Rostock University Medical Center, Schillingallee 35, Rostock 18057, Mecklenburg-Vorpommern, Germany. michael.linnebacher@med.uni-rostock.de
Received: November 10, 2021 Peer-review started: November 10, 2021 First decision: January 9, 2022 Revised: January 17, 2022 Accepted: April 29, 2022 Article in press: April 29, 2022 Published online: May 27, 2022
Abstract
Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are a rare group of tumors originating from neuroendocrine cells of the digestive system. Their incidence has increased over the last decades. The specific pathogenetic mechanisms underlying GEP-NEN development have not been completely revealed. Unfunctional GEP-NENs are usually asymptomatic; some grow slowly and thus impede early diagnosis, which ultimately results in a high rate of misdiagnosis. Therefore, many GEP-NEN patients present with later staged tumors. Motivated hereby, research attention for diagnosis and treatment for GEP-NENs increased in recent years. The result of which is great progress in clinical diagnosis and treatment. According to the most recent clinical guidelines, improved grading standards can accurately define poorly differentiated grade 3 neuroendocrine tumors and neuroendocrine carcinomas (NECs), which are subclassified into large and small cell NECs. Combining different functional imaging methods facilitates precise diagnosis. The expression of somatostatin receptors helps to predict prognosis. Genetic analyses of mutations affecting death domain associated protein (DAXX), multiple endocrine neoplasia type 1 (MEN 1), alpha thalassemia/intellectual disability syndrome X-linked (ATRX), retinoblastoma transcriptional corepressor 1 (RB 1), and mothers against decapentaplegic homolog 4 (SMAD 4) help distinguishing grade 3 NENs from poorly differentiated NECs. The aim of this review is to summarize the latest research progress on diagnosis and treatment of GEP-NENs.
Core Tip: Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are a group of heterogeneous tumors arising from neuroendocrine cells of the digestive system. Researchers have achieved great improvements in diagnosis and treatment. This includes improved grading, identification of specific genetic mutations, functional imaging, and broad application of peptide receptor radionuclide therapy. Here, we systematically summarized the latest progress in diagnosis and treatment of GEP-NENs, thereby providing guidance for clinicians active in this field.
