Zollinger-Ellison syndrome

It is difficult to accurately predict the prevalence and the incidence of Zollinger-Ellison syndrome (ZES), because the use of proton-pump inhibitors can mask the symptoms of acid hypersecretion, preventing diagnosis.[6]Corleto DV, Annibale B, Gibril F, et al. Does the widespread use of proton pump inhibitors mask, complicate and/or delay the diagnosis of Zollinger-Ellison syndrome? Aliment Pharmacol Ther. 2001 Oct;15(10):1555-61. http://onlinelibrary.wiley.com/doi/10.1046/j.1365-2036.2001.01085.x/full http://www.ncbi.nlm.nih.gov/pubmed/11563994?tool=bestpractice.com It is estimated to occur in 1 to 3 patients per million per year in the US, and is believed to be responsible for 1% of duodenal ulcers.

Diagnosis typically occurs between the ages of 20 and 50 years, but has occurred as young as 9 years and as old as 90 years. There is equal distribution among men and women.

About 20% of patients with ZES have multiple endocrine neoplasia type 1; the remaining cases are sporadic.[3]Norton JA. Neuroendocrine tumors of the pancreas and duodenum. Curr Probl Surg. 1994 Feb;31(2):77-156. http://www.ncbi.nlm.nih.gov/pubmed/7904550?tool=bestpractice.com

Progression to metastatic gastrinoma is the most common cause of mortality. Complications resulting from peptic ulcer disease used to be the most common cause of death, but these have been significantly reduced because of the widespread use of proton-pump inhibitor therapy.