What is Zollinger-Ellison syndrome (ZES)?
ZES is a rare disorder characterized by one or more tumors in the
pancreas, duodenum, or both. The tumors cause the stomach to make
too much acid, leading to peptic ulcers in the duodenum. The
tumors are sometimes cancerous and spread to other areas of the
body.
What are the stomach, duodenum, and
pancreas?
The stomach, duodenum, and pancreas are digestive organs. The
stomach produces gastric acid and other digestive juices that
break down food. Partially digested food moves into the duodenum
and is further broken down. The duodenum is the first part of the
small intestine—the tube-shaped organ between the stomach and the
large intestine. The pancreas is a large gland that produces
digestive juices that flow into the duodenum. The pancreas also
makes hormones that are released into the bloodstream. Hormones
are powerful chemicals produced by glands that control the
function of cells and organs.
What causes ZES?
ZES is caused by tumors called gastrinomas, which release the
hormone gastrin.
Normally, cells in the stomach produce and control gastrin so only
the right amount is released. Gastrin travels through the
bloodstream to signal other cells in the stomach to release
gastric acid to help break down food. Gastrinomas release abnormal
amounts of gastrin, resulting in excess gastric acid in the
stomach and duodenum. The excess acid eventually causes sores
called peptic ulcers to form in the lining of the duodenum.
Scientists are unsure what causes the majority of gastrinomas,
which appear sporadically. About 25 percent of gastrinoma cases
are caused by an inherited genetic disorder called multiple
endocrine neoplasia type 1 (MEN1).1 MEN1 can cause a variety of
hormone-releasing tumors such as prolactinomas and insulinomas.
Prolactinomas form in the pituitary gland in the brain and cause
excess prolactin—a hormone that influences milk production,
fertility, and bone strength. Insulinomas form in the pancreas and
cause excess insulin—a hormone that helps control blood glucose,
also called blood sugar. Signs and symptoms of MEN1 include
increased hormone levels in the blood, kidney stones, diabetes,
muscle weakness, and weakened bones and fractures.
What are the symptoms of ZES? ZES symptoms are similar to those of peptic ulcers and include
burning abdominal pain
nausea and vomiting
weight loss
diarrhea
severe gastroesophageal reflux—a condition where gastric acid and
food from the stomach backs up into the esophagus
How is ZES treated?
ZES is treated with medications to relieve ulcer symptoms and
surgery, if appropriate, to remove tumors. Chemotherapy is
sometimes used when tumors are too widespread to remove with
surgery.
A class of drugs called proton pump inhibitors effectively
reduces gastric acid secretion in the stomach and includes
esomeprazole (Nexium)
lansoprazole (Prevacid)
pantoprazole (Protonix)
omeprazole (Prilosec)
Reducing stomach acid allows peptic ulcers to heal and relieves
ZES symptoms.
Surgical removal of gastrinomas is the only cure for ZES. Some
gastrinomas behave like cancers and spread to other parts of the
body, especially the liver and bones. Finding and removing all
gastrinomas is often challenging.
Gastrinomas that cannot be surgically removed are sometimes
treated with chemotherapy drugs, including
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