Zollinger-Ellison syndrome screening

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]; Aravind Reddy Kothagadi M.B.B.S[3]

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Overview

Measurement of fasting serum gastrin levels is the single best screening test for Zollinger-Ellison syndrome (ZES). Other tests such as provocative tests include, the secretin stimulation test, calcium stimulation test, secretin-plus-calcium stimulation tests, bombesin test, and protein meal test.

Screening

  • Measurement of fasting serum gastrin levels is the single best screening test for Zollinger-Ellison syndrome (ZES).
  • It is advised not to take gastric antisecretory medications at the time of the test.
  • Serial multiple measurements on different days have been advised as the fasting gastrin levels can fluctuate from day to day and may appear to be normal.
  • In untreated ZES, normal levels of serum gastrin are extremely rare (<1%).
  • Multiple endocrine neoplasia-type 1 (MEN 1) syndrome should be suspected if serum calcium levels are elevated greater than 12.0 mg/dl accompanied with gastrinoma.
    • Gastric acid secretory test: ZES is suspected in patients with a basal acid output (BAO) is greater than 15 mEq/h. ZES is also suspected in patients with a prior vagotomy and partial gastrectomy whose basal acid output (BAO) is greater than 5 mEq/h.
      • Basal gastric secretory volume greater than 140 mL is considered highly sensitive and specific in patients without any prior gastric acid lowering surgery,
      • Gastric acid pH less than 2.0 in the presence of a large gastric volume greater than 140 mL over 1 hour is highly suggestive of ZES in patients without any prior gastric acid lowering surgery,
    • Provocative tests: [1]
  • Secretin stimulation test is the provocative test of choice because of its higher sensitivity. In this test, a 2-U/kg bolus of secretin is administered intravenously after an overnight fast, and serum levels of gastrin are determined at 0, 2, 5, 10, and 15 minutes. An increase in serum gastrin of greater than 200 pg/mL is diagnostic.
  • Imaging studies such as Somatostatin receptor scintigraphy (SRS) is helpful in staging and localizing the gastrinoma.
  • Screening also helps in determining if the patient is fit to undergo surgery for tumor resection.

References

  1. Berna MJ, Hoffmann KM, Long SH, Serrano J, Gibril F, Jensen RT (2006). "Serum gastrin in Zollinger-Ellison syndrome: II. A prospective study of gastrin provocative testing in 293 patients from the National Institutes of Health and comparison with 537 cases from the literature. evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features". Medicine (Baltimore). 85 (6): 331–64. doi:10.1097/MD.0b013e31802b518c. PMID 17108779.
  2. Shah P, Singh MH, Yang YX, Metz DC (2013). "Hypochlorhydria and achlorhydria are associated with false-positive secretin stimulation testing for Zollinger-Ellison syndrome". Pancreas. 42 (6): 932–6. doi:10.1097/MPA.0b013e3182847b2e. PMC 3712291. PMID 23851430.

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