Zollinger-Ellison syndrome

Last revised by Sonam Vadera on 16 Oct 2022

Zollinger-Ellison syndrome (ZES) is a clinical syndrome that occurs secondary to a gastrinoma

Diagnosis of Zollinger-Ellison syndrome is often delayed by 5-7 years after the onset of symptoms 2

Gastrinomas are usually multiple and typically located in the duodenum (more common) or pancreas (less common). These tumors secrete gastrin that results in hypersecretion of gastric acid, which in turn results in diarrhea, gastritis, severe gastro-esophageal reflux disease and peptic ulcer disease 1

  • increased gastrin levels in fasting patients (but not specific, and some data suggest that clinical assays may be unreliable 6

On double-contrast upper gastrointestinal studies the following features may be seen 4,5:

  • thickened rugal folds
  • multinodular gastric contour
  • erosions and ulcers, especially in atypical locations
  • barium may be diluted by the high volume of fluid in the stomach
  • negative contrast may be used to distend the stomach
  • thickened rugal folds
  • multiple gastric nodules/masses 4

Lesions show increased uptake on somatostatin receptor scintigraphy using 111In-labeled octreotide and somatostatin receptor PET/CT using 68Ga-DOTANOC 9.

Surgery plays a vital role 8. Death from complications of Zollinger-Ellison syndrome (e.g. perforation, hemorrhage) can occur. 

It is named after Robert M Zollinger and Edwin H Ellison, who in April 1956 described two cases of severe, multifocal ulcerative lesions of the proximal gastrointestinal tract, which were remittent, refractory to surgery, and associated with tumors in the adjacent pancreas 7

Possible differential considerations include:

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Cases and figures

  • Case 1a: on CT
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  • Case 1b: on PET-CT
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