Rikhraj N, Fernandez CJ, Ganakumar V, Pappachan JM. Pancreatic neuroendocrine tumors: A case-based evidence review. World J Gastrointest Pathophysiol 2025; 16(2): 107265 [DOI: 10.4291/wjgp.v16.i2.107265]
Corresponding Author of This Article
Joseph M Pappachan, MD, MRCP, FRCP, Professor, Senior Researcher, Faculty of Science, Manchester Metropolitan University, Manchester, Manchester M15 6BH, Greater Manchester, United Kingdom. drpappachan@yahoo.co.in
Research Domain of This Article
Endocrinology & Metabolism
Article-Type of This Article
Evidence 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 Pathophysiol. Jun 22, 2025; 16(2): 107265 Published online Jun 22, 2025. doi: 10.4291/wjgp.v16.i2.107265
Pancreatic neuroendocrine tumors: A case-based evidence review
Naveena Rikhraj, Cornelius J Fernandez, Vanishri Ganakumar, Joseph M Pappachan
Naveena Rikhraj, Department of Medicine, Lancashire Teaching Hospitals NHS Trust, Preston PR2 9HT, Lancashire, United Kingdom
Cornelius J Fernandez, Department of Endocrinology and Metabolism, Pilgrim Hospital, United Lincolnshire Hospitals NHS Trust, Boston PE21 9QS, Lincolnshire, United Kingdom
Vanishri Ganakumar, Department of Endocrinology, Jawaharlal Nehru Medical College, Belagavi 590010, Karnātaka, India
Joseph M Pappachan, Faculty of Science, Manchester Metropolitan University, Manchester M15 6BH, United Kingdom
Joseph M Pappachan, Department of Endocrinology and Metabolism, Kasturba Medical College, Manipal & Manipal Academy of Higher Education, Manipal 576104, India
Author contributions: Rikhraj KN and Fernandez CJ performed the initial drafting of article, designed all figures and performed formal analysis of literature and review; Ganakumar V and Pappachan JM conceived the idea for drafting and provided additional input on the review process; Pappachan JM provided overall supervision and editing. All the authors reviewed and approved the revision of the paper.
Conflict-of-interest statement: There are no conflicts in interest among authors in relation to this manuscript.
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: Joseph M Pappachan, MD, MRCP, FRCP, Professor, Senior Researcher, Faculty of Science, Manchester Metropolitan University, Manchester, Manchester M15 6BH, Greater Manchester, United Kingdom. drpappachan@yahoo.co.in
Received: March 19, 2025 Revised: April 13, 2025 Accepted: May 18, 2025 Published online: June 22, 2025 Processing time: 92 Days and 10.6 Hours
Abstract
Pancreatic neuroendocrine tumors (pNETs) are rare, presenting significant challenges in timely diagnosis and subsequent treatment. The clinical and pathobiological behavior of these tumors varies significantly, making follow-up and therapeutic approaches challenging for clinicians. Although the majority of these neoplasms are hormonally inactive, some can be associated with endocrine dysfunction. Very rarely, a nonfunctional tumor can later become hormonally active, further complicating prognostication and management. Depending on the character of the disease, clinical picture and prognosis, different treatment modalities are instituted with varying effectivities. We recently came across a unique case of nonfunctioning malignant pNET at an advanced stage, metastatic disease upon diagnosis, managed medically with somatostatin analog therapy (Octreotide) and targeted therapy (Everolimus) with stable disease for 40 months that subsequently turned out to become functional (insulinoma). With the aid of this unique case, we update the current clinical, diagnostic and therapeutic approach to pNETs in this evidence-based review.
Core Tip: Pancreatic neuroendocrine tumors (pNETs) are rare neoplasms with variable clinical and pathobiological characteristics posing diagnostic and therapeutic challenges. Mostly nonfunctional with low growth rates, some cases can be functional while others can run an aggressive clinical course. Hormonal, imaging and histological assessments are essential for planning appropriate diagnosis, prognosis and management. Therapeutic strategies depend on the tumor grade, functionality and presence of metastatic disease. Nonfunctioning pNETs very rarely turn functional during the advanced disease course. Presenting a unique nonfunctioning metastatic pNET case, later evolving to an insulinoma, we update the diagnostic, prognostic and management strategies for pNETs in the evidence-based review.
