Insulinoma is a topic covered in the 5-Minute Clinical Consult.

To view the entire topic, please or purchase a subscription.

Medicine Central™ is a quick-consult mobile and web resource that includes diagnosis, treatment, medications, and follow-up information on over 700 diseases and disorders, providing fast answers—anytime, anywhere. Explore these free sample topics:

Medicine Central

-- The first section of this topic is shown below --

Basics

Description

  • Neoplasm of the pancreatic β cells of the islets of Langerhans leading to hyperinsulinemia. Pancreatic β cells manufacture proinsulin, which is then cleaved into insulin and C-peptide before secretion.
  • Most common functional (insulin-producing) tumor of the pancreas
  • 90% are located within the pancreas.
  • Usually small with 90% <2 cm; suspect malignant insulinoma if tumor is >4 cm.
  • Symptoms due to hypoglycemia and concomitant cerebral glucose deprivation
  • Diagnosis of insulinoma is often delayed due to vague and unusual presentation of neuroglycopenic symptoms.
  • Hypoglycemic episodes are irregular, recurrent, and tend to increase in frequency and severity over time.
  • Definitive management consists of surgical resection.
  • Synonym(s): β-cell tumor; β-cell adenoma; nesidioblastoma

Epidemiology

Incidence
  • 3 to 4 cases per 1 million person-years
  • 10% malignant, 10% multiple tumors (1)
  • Two major categories: sporadic (>90%) and multiple endocrine neoplasia type 1 (MEN1) syndrome (5–10%); etiology dictates treatment approach (2).
    • Sporadic type tends to be solitary and benign; sporadic type associated with slight female predominance (3)
    • When insulinoma associated with MEN1, usually accompanied by multiple lesions; patients typically present in the 3rd decade of life without significant gender-specific incidence (2).

Prevalence
The prevalence of insulinoma is 0.4% (4).

Genetics
  • Mutations in DAXX and ATRX have been identified in 43% of pancreatic endocrine tumors (4).
  • MEN syndrome associated with approximately 10% of cases; result of mutation in menin gene on long arm of chromosome 11 (2,5)

Risk Factors

MEN1 syndrome only known risk factor (1)

Commonly Associated Conditions

MEN1 syndrome (1)

-- To view the remaining sections of this topic, please or purchase a subscription --

Citation

* When formatting your citation, note that all book, journal, and database titles should be italicized* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Insulinoma ID - 816207 ED - Baldor,Robert A, ED - Domino,Frank J, ED - Golding,Jeremy, ED - Stephens,Mark B, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816207/all/Insulinoma PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -