Zollinger-Ellison syndrome screening
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%)
- If serum calcium levels are elevated, multiple endocrine neoplasia-type 1 (MEN 1) syndrome should be suspected.
- Gastric acid secretory test: In patients with a prior vagotomy and partial gastrectomy,basal acid output (BAO) greater than 15 mEq/h or greater than 5 mEq/h is suggestive of ZES.
- In patients with no prior gastric acid–reducing surgery, basal gastric secretory volume greater than 140 mL has a high sensitivity and specificity.
- In patients without prior gastric acid–reducing surgery), gastric pH less than 2.0 in the presence of a large gastric volume (>140 mL over 1 h is highly suggestive of ZES.
- 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.
- Evaluation of a patient with suspected gastrinoma:
- Step 1: Fasting gastrin levels are evaluated. A minimum of 3 fasting levels of gastrin on different days are measured.
- Step 2: Gastric acid secretory studies which reveal highly suggestive findings of gastrinoma:
- Basal acid output (BAO) value of greater than 15 mEq/h (OR) [3]
- Gastric volume of greater than 140 mL and pH of less than 2.0 .
- Step 3: Perform a provocative test. The secretin stimulation test is the preferred test.
- Step 4: Perform somatostatin receptor scintigraphy (SRS).
- Step 5: Perform imaging studies to stage and localize the gastrinoma.
- Step 6: Determine if patient is a surgical candidate for tumor resection.
References
- ↑ Berna MJ, Hoffmann KM, Long SH, Serrano J, Gibril F, Jensen RT (2006). "Serum gastrin in Zollinger-Ellison syndrome: II. 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.
- ↑ 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.
- ↑ Roy PK, Venzon DJ, Feigenbaum KM, Koviack PD, Bashir S, Ojeaburu JV, Gibril F, Jensen RT (2001). "Gastric secretion in Zollinger-Ellison syndrome. Correlation with clinical expression, tumor extent and role in diagnosis--a prospective NIH study of 235 patients and a review of 984 cases in the literature". Medicine (Baltimore). 80 (3): 189–222. PMID 11388095.