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Strongly supportive of ZES diagnosis
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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.
a positive biopsy or cytology for a neuroendocrine tumor (NET) (stronger support if a gastrinoma is found)
a positive secretin test
known or strongly suspected MEN1 syndrome (i.e., a positive family history, hyperparathyroidism or pituitary disease)
A patient with known MEN1 or strongly suspected MEN1 (i.e., a positive family history, hyperparathyroidism or pituitary disease) with a positive gastrinoma by cytology/biopsy
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Moderately supportive of ZES diagnosis (consider this a tentative diagnosis)
Positive somatostatin receptor scintigraphy imaging (SRI) with either 68Ga-DOTATATE PET/CT or 111In-DTPA-octreotide with SPECT/CT imaging (sporadic disease only) or positive cytology or biopsy for a NET, ideally a gastrinoma, (sporadic disease or MEN1 syndrome present) with a biopsy-proven absence of atrophic gastritis and negative autoimmune markers. (1,2).
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Weakly supportive of ZES diagnosis (insufficient alone for even a tentative diagnosis)
A patient with known MEN1 or strongly suspected MEN1 (i.e., a positive family history, hyperparathyroidism or pituitary disease) with positive imaging or an SRI (1)
MEN1 syndrome absent but positive SRI or imaging for possible tumor (3).
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