Challenges in diagnosing and treating pancreatic neuroendocrine tumours in patients with a multiple endocrine neoplasia type 1 (MEN1) syndrome Review article
Article Sidebar
Main Article Content
Abstract
Multiple endocrine neoplasia type 1 (MEN1) syndrome is an autosomal dominant hereditary disorder characterised by coexistence of pancreatic neuroendocrine tumours (pNETs) with parathyroid and pituitary tumours. PNETs, including mostly non-functioning tumours, gastrinoma and insulinoma, occur in nearly 95% of MEN1 patients and account for over 50% of disorder-related mortality. Therefore, early initiation of screening for pNET using biochemical and imaging tests as well as appropriate surgical and systemic treatment are of particular importance for this group of patients. Currently, there are no clearly defined guidelines which determine the optimal methods for detection and treatment of pNET in MEN1. Caution should be exercised when applying the guidelines designed for patients with sporadic pNET to MEN1 patients as the clinical course of the disorder is slightly different, involving multifocality of lesions and younger age of patients at onset. This paper discusses the distinctive features and challenges in diagnosing and treating pNETs in MEN1 patients.
Downloads
Metrics
Article Details
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Copyright: © Medical Education sp. z o.o. This is an Open Access article distributed under the terms of the Attribution-NonCommercial 4.0 International (CC BY-NC 4.0). License (https://creativecommons.org/licenses/by-nc/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
Address reprint requests to: Medical Education, Marcin Kuźma (marcin.kuzma@mededu.pl)
References
2. Yates CJ, Newey PJ, Thakker RV. Challenges and controversies in management of pancreatic neuroendocrine tumours in patients with MEN1. Lancet Diabetes Endocrinol 2015; 3(11): 895-905.
3. Gietka-Czernel M, Kozakowski J, Szczepańska E. Zespół mnogich nowotworów gruczołów dokrewnych typu 1 (MEN1). Post N Med 2017; XXX(12): 665-673.
4. Amin S, Kang Kim M. Islet Cell Tumors of the Pancreas. Gastroenterol Clin North Am 2016; 45(1): 83-100.
5. Kos-Kudła B, Rosiek V, Borowska M et al. Pancreatic neuroendocrine neoplasms – management guidelines (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol 2017; 68(2): 169-197.
6. Ito T, Igarashi H, Uehara H et al. Causes of death and prognostic factors in multiple endocrine neoplasia type 1: a prospective study: comparison of 106 MEN1/Zollinger-Ellison syndrome patients with 1613 literature MEN1 patients with or without pancreatic endocrine tumors. Medicine (Baltimore) 2013; 92(3): 135-181.
7. Newey PJ, Jeyabalan J, Walls GV et al. Asymptomatic children with multiple endocrine neoplasia type mutations may harbor nonfunctioning pancreatic neuroendocrine tumors. J Clin Endocrinol Metab 2009; 94(10): 3640-3646.
8. Falconi M, Ericsson B, Kaltsas G et al. ENETS Consensus Guidelines Update for the Management of Patients with Functional Pancreatic Neuroendocrine Tumors and Non-Functional Pancreatic Neuroendocrine Tumors. Neuroendocrinology 2016; 103: 153-171.
9. de Laat JM, Pieterman CR, Weijmans M et al. Low accuracy of tumor markers for diagnosing pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1 patients. J Clin Endocrinol Metab 2013; 98(10): 4143-4151.
10. Qiu W, Christakis I, Silva A et al. Utility of chromogranin A, pancreatic polypeptide, glucagon and gastrin in the diagnosis and follow-up of pancreatic neuroendocrine tumours in multiple endocrine neoplasia type 1 patients. Clin Endocrinol (Oxf) 2016; 85(3): 400-407.
11. Oberg K, Modlin IM, De Herder W et al. Consensus on biomarkers for neuroendocrine tumour disease. Lancet Oncol 2015; 16(9): e435-e446.
12. Langer P, Kann PH, Fendrich V et al. Prospective evaluation of imaging procedures for the detection of pancreaticoduodenal endocrine tumors in patients with multiple endocrine neoplasia type 1. World J Surg 2004; 28(12): 1317-1322.
13. Camera L, Paoletta S, Mollica C et al. Screening of pancreaticoduodenal endocrine tumours in patients with MEN 1: multidetector-row computed tomography vs. endoscopic ultrasound. Radiol Med 2011; 116(4): 595-606.
14. Barbe C, Murat A, Dupas B. Magnetic resonance imaging versus endoscopic ultrasonography for the detection of pancreatic tumours in multiple endocrine neoplasia type 1. Dig Liver Dis 2012; 44(3): 228-234.
15. Harper S, Harrison B. First surgery for pancreatic neuroendocrine tumours in a patient with MEN1: enucleation versus disease-modifying surgery. Clin Endocrinol (Oxf) 2015; 83(5): 618-621.
16. Triponez F, Dosseh D, Goudet P et al. Epidemiology data on 108 MEN1 patients from the GTE with isolated nonfunctioning tumors of the pancreas. Ann Surg 2006; 243: 265-272.
17. Pai M, Habib N, Senturk H et al. Endoscopic ultrasound guided radiofrequency ablation, for pancreatic cystic neoplasms and neuroendocrine tumors. World J Gastrointest Surg 2015; 7(4): 52-59.
18. Lakhtakia S. Therapy of Pancreatic Neuroendocrine Tumors: Fine Needle Intervention including Ethanol and Radiofrequency Ablation. Clin Endosc 2017; 50(6): 546-551.
19. Dąbkowski K, Gajewska P, Walter K et al. Successful EUS-guided ethanol ablation of insulinoma, four-year follow-up. Case report and literature review. Endokrynol Pol 2017; 68(4): 472-479. DOI: 10.5603/EP.2017.0053.
20. Kos-Kudła B, Blicharz-Dorniak J, Strzelczyk J et al. Diagnostic and therapeutic guidelines for gastro-entero pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol 2017; 68(2): 79-110.
21. Ramundo V, Del Prete M, Marotta V et al. Impact of long-acting octreotide in patients with early-stage MEN1 related duodeno-pancreatic neuroendocrine tumours. Clin Endocrinol (Oxf) 2014; 80(6): 850-865.