Diagnostic criteria for ZES include the following:

1. Elevated levels of Basal Acid Output (BAO), greater than 15 mEq in unoperated patients and greater than 5 mEq if previous acid-reducing surgery has been performed;
2. Elevated level of fasting serum gastrin (>100 pg/mL until 1994, >200 pg/mL since 1994);
3. Abnormal results from stimulation testing with secretin (an increase of >200 pg/mL postinjection) or with calcium (an increase >395 pg/mL);
4. Positive histologic confirmation of gastrinoma; or
5. A combination of these criteria.

The tests used most commonly to establish a diagnosis of ZES are the fasting serum gastrin concentration and BAO evaluation. Measurement of gastric pH is important to exclude achlorhydria as a cause of secondary hypergastrinemia. Patients taking Proton Pump Inhibitors (PPIs), those who have undergone massive small bowel resection, or those who have renal insufficiency, G-cell hyperplasia, or gastric outlet obstruction may have gastrin levels between 150 and 1000 pg/mL Hence, for patients with suspected ZES with an equivocal fasting serum gastrin concentration, a secretin stimulation test should be performed.

 

References:

1. Gibril F, Schumann M, Pace A, Jensen RT. Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature. Medicine (Baltimore). 2004;83:43-83. [Medline]