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. Author manuscript; available in PMC: 2018 Apr 11.
Published in final edited form as: Int J Endocr Oncol. 2017 Oct 11;4(4):167–185. doi: 10.2217/ije-2017-0018

Table 4.

Possible new criteria supporting the diagnosis of ZES in patients with Fasting Hypergastrinemia taking PPIs (1)

  1. Moderately supportive of ZES diagnosis (ZES is likely)

    1. In a patient with or without MEN1 with active peptic ulcer disease (PUD) or a history compatible with recent PUD or improvement in diarrhea with PPIs combined with a positive biopsy or cytology for a neuroendocrine tumor (NET) (stronger support if a gastrinoma is found.

    2. In a patient without MEN1 with active peptic ulcer disease (PUD) or a history compatible with recent PUD or improvement in diarrhea with PPIs combined with a positive somatostatin receptor scintigraphy imaging (SRI) with either 68Ga-DOTATATE PET/CT or 111In-DTPA-octreotide with SPECT/CT imaging. (2).

  2. Weakly supportive of ZES diagnosis (consider this a tentative diagnosis)

    1. In a patient without active PUD or history of diarrhea responding to PPIs without MEN1 with a biopsy-proven absence of atrophic gastritis and negative autoimmune markers with a positive SRI (3,4)

    2. In a patient without active PUD or history of diarrhea responding to PPIs with known MEN1 or strongly suspected MEN1 (i.e., a positive family history, hyperparathyroidism or pituitary disease) with a biopsy-proven absence of atrophic gastritis. (3) and negative autoimmune markers. (4)

  3. Minimally supportive of ZES diagnosis (consider this a possible diagnosis only)

    1. In a patient without active PUD or history of diarrhea responding to PPIs without MEN1 with a positive SRI

    2. A patient with known MEN1 or strongly suspected MEN1 (i.e., a positive family history, hyperparathyroidism or pituitary disease) without active PUD or history of diarrhea responding to PPIs with prominent gastric folds (5).

(1)

The potential for a false-positive secretin test in patients with hypo-/achlorhdria limits the usefulness of the secretin test in patients taking PPIs unless the gastric pH≤2.

(2)

Under these conditions a NET is likely but since MEN1 patients develop multiple NETs in various locations NET(s) a positive SRI or biopsy may not be a gastrinoma(s) [8, 18, 33, 162]

(3)

Five biopsies (2-antrum, 2-corpus,1-incisura angularis) of the stomach are recommended to diagnose atrophic gastritis)[124, 125].

(4)

Biopsy and autoimmune markers can both be negative in confirmed autoimmune gastropathy [124, 125].

(5)

Prominent gaastric folds are present in 92% of ZES patients when initially seen, however they are not specfic for ZES [2]