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 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: ZES occurs when 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

Treatment and prognosis

Surgery plays a vital role ⁸. Death from complications of ZES (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 
• previous vagotomy
• pernicious anemia ⁸ 
• Ménétrier disease