First described by Zollinger and Ellison in 1955. Syndrome results from excess gastrin stimulated acid production

Classic Triad

• Fulminating gastric ulcers
• Recurrent ulceration despite therapy
• Non - beta cell pancreatic islet cell tumour

Incidence

• 1:1000 000 per year
• 50% have metastases at diagnosis
• 60% are malignant
• 30% MEN 1 (tend to be multifocal)
• Majority are pancreatic
• 20 - 40% Duodenal microgastrinomas

Clinical Features

• Peptic Ulceration - multiple, atypical sites
• Diarrhoea - severe in 40% (diarrhoea without ulceration in 7%)
• Steatorrhoea
• Enzyme inactivation ( pancreatic lipase)
• Mucosal damage

Diagnosis

• GASTRIN > 40pmol/l
• Fasted sample
• Off antisecretory medication (proton pump inhibitors for at least 2 weeks and H2 antagonists for at least 3 days)
• Measure Gastric Acid Secretion - If Doubt About Diagnosis

Localisation

• >90% of gastrinomas are found in the Gastrinoma Triangle:

1. third part of the duodenum
2. neck of the pancreas
3. the porta hepatis

• 20% in the duodenum
• CT and visceral angiography (+/- calcium/ secretin stimulation) will localise 70%

Treatment

• Reduction in Tumour Load
• Surgery
• Hepatic embolisation
• Proton Pump Inhibitors
• Labile in acid therefore should be given initially with H2 blocker
• Octreotide