Zollinger-Ellison syndrome (ZES) is a clinical syndrome that occurs secondary to elevated gastrin levels produced by a gastrinoma. 

Clinical presentation

The syndrome presents with abdominal pain but can also present with diarrhoea or other complaints as well. Diagnosis is often delayed by 5-7 years after the onset of symptoms ². 

Pathology

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

Markers

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

Associations

• multiple endocrine neoplasia (MEN) type 1: Zollinger-Ellison syndrome occurs when the gastrinoma is functional

Radiographic features

Fluoroscopy

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

CT

• negative contrast may be used to distend the stomach

• thickened rugal folds

• multiple gastric nodules/masses ⁴

Nuclear medicine

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

Treatment and prognosis

Surgery plays a vital role ⁸. Death from complications of Zollinger-Ellison syndrome (e.g. perforation, haemorrhage) 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 tumours in the adjacent pancreas ⁷. 

Differential diagnosis

Possible differential considerations include:

• gastritis from other causes, e.g. H. pylori infection, hypertrophic gastritis

• gastric lymphoma ⁵  

• gastric outlet obstruction 

• peptic ulcer disease of other aetiology

• previous vagotomy

• pernicious anaemia ⁸ 

• Ménétrier disease