Zollinger-Ellison Syndrome

Zollinger-Ellison Syndrome is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • Zollinger-Ellison syndrome (ZES) triad
    • Markedly elevated gastric acid secretion
    • Peptic ulcer disease
    • A gastrinoma or non-β islet cell tumor of the pancreas or duodenal wall that produces gastrin
      • Gastrinomas (at the time of diagnosis) may be single or multiple (1/2 to 2/3), large or small, benign or malignant (2/3), sporadic (70–75%) or associated with multiple endocrine neoplasia type 1 (MEN1) (25–30%).
  • System(s) affected: endocrine/metabolic, gastrointestinal
  • Synonym(s): Z-E syndrome; pancreatic ulcerogenic tumor syndrome; multiple endocrine neoplasia, partial; ulcerogenic islet cell tumor

Epidemiology

Incidence
  • 1 to 3 per million per year in the United States
  • Predominant age: middle age (30 to 65 years). Mean age of onset is 43 years; presents a decade earlier in patients with ZES/MEN1
  • Predominant sex: male > female (1.3:1)

Pediatric Considerations
Aggressive cases have been reported in teenagers.

Pregnancy Considerations
Rare, pregnancy alters medication choices and surgical timing.

Etiology and Pathophysiology

  • Gastrinoma is equally distributed between the head of the pancreas and the first or second portion of the duodenum; if in the pancreas, the lesion is more likely to metastasize to the liver.
  • Hypergastrinemia results in gastric mucosal hypertrophy and increased acid production. Increased acid production causes mucosal ulceration. Diarrhea and malabsorption are also common in ZES.
  • Increasing number found in stomach wall, up to 8%; may be due to increased surveillance and/or increased PPI use masking symptoms (1)[C]
  • Also may be found rarely in the mesentery, peritoneum, spleen, skin, or mediastinum (possibly metastasis with primary not identified)

Genetics
~25–30% of cases occur in association with the MEN1 syndrome—tumors of pancreas, pituitary, and parathyroid.

Risk Factors

  • MEN1
  • Family history of ulcer disease

General Prevention

Screen first-degree relatives of patients with MEN1.

Commonly Associated Conditions

  • MEN1
  • Insulinoma
  • Carcinoid tumors

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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 - Zollinger-Ellison Syndrome ID - 116659 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/116659/all/Zollinger_Ellison_Syndrome PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -