Zollinger-Ellison syndrome (ZES) involves hypergastrinemia produced by
gastrin-secreting
tumor(s) of the pancreas or duodenum. Estimated to occur in 0.1-3 per million of the United States' population, the actual prevalence may be higher because ZES is often undetected with routine testing. ZES should be suspected in patients who present with persistent or complex duodenal or postsurgical
ulcer, especially if accompanied by
esophagitis,
diarrhea,
weight loss, and/or liver
metastases. Twenty percent of ZES patients have
multiple endocrine neoplasia type I, some of whom may also have elevated levels of serum
calcium and a family history of ZES. Diagnostic tests include fasting serum
gastrin concentration, gastric secretion analysis, with, if necessary,
secretin stimulation of serum
gastrin. Complete surgical tumorectomy for cure is impossible in as many as 70-90% of patients with ZES, who then require long-term medical
therapy to reduce
acid exposure. Basal
acid output needs to be maintained at < 5 mEq/h for uncomplicated ZES and at < 1-2 mEq/h for complicated ZES or postgastrectomy.
Proton pump inhibitors (
omeprazole,
lansoprazole) with careful clinical monitoring provide safe and effective
acid control in patients with ZES.