PMID- 37925662 OWN - NLM STAT- MEDLINE DCOM- 20240318 LR - 20240318 IS - 1945-7197 (Electronic) IS - 0021-972X (Print) IS - 0021-972X (Linking) VI - 109 IP - 4 DP - 2024 Mar 15 TI - Approach to the Patient: Insulinoma. PG - 1109-1118 LID - 10.1210/clinem/dgad641 [doi] AB - Insulinomas are hormone-producing pancreatic neuroendocrine neoplasms with an estimated incidence of 1 to 4 cases per million per year. Extrapancreatic insulinomas are extremely rare. Most insulinomas present with the Whipple triad: (1) symptoms, signs, or both consistent with hypoglycemia; (2) a low plasma glucose measured at the time of the symptoms and signs; and (3) relief of symptoms and signs when the glucose is raised to normal. Nonmetastatic insulinomas are nowadays referred to as "indolent" and metastatic insulinomas as "aggressive." The 5-year survival of patients with an indolent insulinoma has been reported to be 94% to 100%; for patients with an aggressive insulinoma, this amounts to 24% to 67%. Five percent to 10% of insulinomas are associated with the multiple endocrine neoplasia type 1 syndrome. Localization of the insulinoma and exclusion or confirmation of metastatic disease by computed tomography is followed by endoscopic ultrasound or magnetic resonance imaging for indolent, localized insulinomas. Glucagon-like peptide 1 receptor positron emission tomography/computed tomography or positron emission tomography/magnetic resonance imaging is a highly sensitive localization technique for seemingly occult, indolent, localized insulinomas. Supportive measures and somatostatin receptor ligands can be used for to control hypoglycemia. For single solitary insulinomas, curative surgical excision remains the treatment of choice. In aggressive malignant cases, debulking procedures, somatostatin receptor ligands, peptide receptor radionuclide therapy, everolimus, sunitinib, and cytotoxic chemotherapy can be valuable options. CI - (c) The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society. FAU - Hofland, Johannes AU - Hofland J AUID- ORCID: 0000-0003-0679-6209 AD - ENETS Center of Excellence, Department of Internal Medicine, Section of Endocrinology, Erasmus MC and Erasmus MC Cancer Institute, 3015 GD Rotterdam, The Netherlands. FAU - Refardt, Julie C AU - Refardt JC AUID- ORCID: 0000-0002-3040-4685 AD - ENETS Center of Excellence, Department of Internal Medicine, Section of Endocrinology, Erasmus MC and Erasmus MC Cancer Institute, 3015 GD Rotterdam, The Netherlands. AD - ENETS Center of Excellence, Division of Endocrinology, Diabetology and Metabolism, University Hospital Basel, CH-4031 Basel, Switzerland. FAU - Feelders, Richard A AU - Feelders RA AUID- ORCID: 0000-0003-2319-391X AD - ENETS Center of Excellence, Department of Internal Medicine, Section of Endocrinology, Erasmus MC and Erasmus MC Cancer Institute, 3015 GD Rotterdam, The Netherlands. FAU - Christ, Emanuel AU - Christ E AUID- ORCID: 0000-0002-5604-4606 AD - ENETS Center of Excellence, Division of Endocrinology, Diabetology and Metabolism, University Hospital Basel, CH-4031 Basel, Switzerland. FAU - de Herder, Wouter W AU - de Herder WW AUID- ORCID: 0000-0003-1463-5165 AD - ENETS Center of Excellence, Department of Internal Medicine, Section of Endocrinology, Erasmus MC and Erasmus MC Cancer Institute, 3015 GD Rotterdam, The Netherlands. LA - eng PT - Journal Article PL - United States TA - J Clin Endocrinol Metab JT - The Journal of clinical endocrinology and metabolism JID - 0375362 RN - 0 (Receptors, Somatostatin) SB - IM MH - Humans MH - *Insulinoma/diagnosis/therapy/complications MH - Receptors, Somatostatin/therapeutic use MH - *Pancreatic Neoplasms/therapy/drug therapy MH - *Hypoglycemia/diagnosis/etiology/therapy MH - *Neuroendocrine Tumors/complications PMC - PMC10940262 OTO - NOTNLM OT - hypoglycemia OT - insulin OT - insulinoma OT - pathology OT - therapy EDAT- 2023/11/05 18:42 MHDA- 2024/03/18 06:42 PMCR- 2023/10/31 CRDT- 2023/11/05 15:04 PHST- 2023/08/22 00:00 [received] PHST- 2024/03/18 06:42 [medline] PHST- 2023/11/05 18:42 [pubmed] PHST- 2023/11/05 15:04 [entrez] PHST- 2023/10/31 00:00 [pmc-release] AID - 7334393 [pii] AID - dgad641 [pii] AID - 10.1210/clinem/dgad641 [doi] PST - ppublish SO - J Clin Endocrinol Metab. 2024 Mar 15;109(4):1109-1118. doi: 10.1210/clinem/dgad641.