Zollinger–Ellison syndrome

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

Clinical presentation

Diagnosis of ZES is often delayed by 5-7 years after the onset of symptoms .

Pathology

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 .

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

Radiographic features

Fluoroscopy

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

  • 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
CT
  • negative contrast may be used to distend the stomach
  • thickened rugal folds
  • multiple gastric nodules/masses

Treatment and prognosis

Surgery plays a vital role . Death from complications of ZES (e.g. perforation, hemorrhage) can occur.

History and etymology

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 .

Differential diagnosis

Possible differential considerations include:

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