Zollinger-Ellison syndrome

Gastrin-producing neuroendocrine tumour that causes gastric acid hypersecretion and peptic ulcer disease.

Can be sporadic or associated with multiple endocrine neoplasia syndrome type 1.

Common presentation includes refractory peptic ulcer disease, abdominal pain, diarrhoea, and gastro-oesophageal disease.

The main goal of treatment is control of gastric hypersecretion with proton-pump inhibitors.

The most common cause of morbidity and mortality is metastatic gastrinoma.

Zollinger-Ellison syndrome (ZES) is a condition caused by a gastrin-secreting tumour that causes hypersecretion of gastric acid leading to ulcer disease. It most commonly presents with abdominal pain (70% to 100%), diarrhoea (37% to 73%), and gastro-oesophageal reflux disease (44%). Less common presentations include weight loss, GI bleeding, nausea, and vomiting.[1]Nieto JM, Pisegna JR. The role of proton pump inhibitors in the treatment of Zollinger-Ellison syndrome. Expert Opin Pharmacother. 2006 Feb;7(2):169-75. http://www.ncbi.nlm.nih.gov/pubmed/16433582?tool=bestpractice.com [2]Hung PD, Schubert ML, Mihas AA. Zollinger-Ellison syndrome. Curr Treat Options Gastroenterol. 2003 Apr;6(2):163-70. http://www.ncbi.nlm.nih.gov/pubmed/12628075?tool=bestpractice.com