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What is Zollinger-Ellison Syndrome?
(Alternate Names: Gastrinoma, ZE Syndrome)
Zollinger-Ellison syndrome (ZES) is a rare disorder that
causes tumors in the pancreas and duodenum and ulcers in the stomach and
duodenum. The pancreas is a gland located behind the stomach. It produces
enzymes that break down fat, protein, and carbohydrates from food, and
hormones like insulin that break down sugar. The duodenum is the first
part of the small intestine.
The tumors secrete a hormone called gastrin that causes the stomach to
produce too much acid, which in turn causes stomach and duodenal ulcers
(peptic ulcers). The ulcers caused by ZES are less responsive to treatment
than ordinary peptic ulcers. What causes people with ZES to develop tumors
is unknown, but approximately 25 percent of ZES cases are associated with
a genetic disorder called multiple endocrine neoplasia type 1, which is
associated with additional disorders.
The symptoms of ZES include signs of peptic ulcers: gnawing, burning pain
in the abdomen; diarrhea; nausea; vomiting; fatigue; weakness; weight
loss; and bleeding. Physicians diagnose ZES through blood tests to measure
levels of gastrin and gastric acid secretion. They may check for ulcers by
doing an endoscopy, which involves looking at the lining of the stomach
and duodenum through a lighted tube. The clinical diagnosis of ZE
syndrome can be difficult to make and is often delayed occurring
late in the disease course. The diagnosis is further complicated by use of
acid blockers including proton pump inhibitors which can mask signs and
symptoms of the disease.
The primary treatment for ZES is medication to reduce the production of
stomach acid. Proton pump inhibitors that suppress acid production and
promote healing are the first line of treatment and include lansoprazole,
omeprazole, pantoprazole, and rabeprazole. H-2 blockers such as cimetidine,
famotidine, and ranitidine may also be used, but are less effective in
reducing stomach acid. Surgery to treat peptic ulcers or to remove tumors
in the pancreas or duodenum are other treatment options. People who have
been treated for ZES should be monitored in case the ulcers or tumors
recur. (National Organization for Rare Disorders Inc.)
Z-E Publication
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