Approach to the Patient: Insulinoma

Johannes Hofland, Julie C Refardt, Richard A Feelders, Emanuel Christ, Wouter W de Herder*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

1 Citation (Scopus)
34 Downloads (Pure)

Abstract

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.

Original languageEnglish
Article numberdgad641
Pages (from-to)1109-1118
Number of pages10
JournalJournal of Clinical Endocrinology and Metabolism
Volume109
Issue number4
Early online date31 Oct 2023
DOIs
Publication statusPublished - Apr 2024

Bibliographical note

Publisher Copyright: © The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society.

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