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 | Overview |
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Zollinger-Ellison syndrome is a condition in which one or more tumors form in the pancreas or in the upper part of the small intestine (duodenum). These tumors, called gastrinomas, secrete large amounts of the hormone gastrin, which causes excessive production of acid by your stomach, leading to peptic ulcers.
The ulcers that occur as part of Zollinger-Ellison syndrome are typically more resistant to treatment than are other ulcers, can recur after initial treatment, are often numerous and may occur in unusual areas of your stomach or intestine.
Zollinger-Ellison syndrome is rare. In fact, it's estimated that fewer than three out of every million Americans have Zollinger-Ellison. It may occur at any age, but the average age at diagnosis is 50.
Treatment consists of medications to reduce acid and heal the ulcers and, if possible, surgery to remove the tumors.
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| Causes |
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Doctors don't know exactly what causes Zollinger-Ellison syndrome. But the sequence of events in Zollinger-Ellison syndrome is clear. The syndrome begins when a tumor (gastrinoma) or tumors form in your pancreas or duodenum.
Your pancreas is located behind and below your stomach. It produces enzymes that are essential to digesting food. The pancreas also produces several hormones, including insulin and glucagon, both of which regulate your level of blood sugar, along with the stomach hormone gastrin, which controls production of stomach acid. The duodenum, the upper part of the small intestine, begins at the lower end of your stomach. In the duodenum, digestive juices from the pancreas, liver and gallbladder mix and digestion reaches its peak.
The tumors are made up of cells that secrete very large amounts of gastrin, which in turn causes excessive acid production in your stomach. This excessive acid production then causes peptic ulcers.
As well as leading to ulcers, the gastrinomas may sometimes be cancerous (malignant). Gastrinomas grow slowly, but the cancer can spread elsewhere — usually to nearby lymph nodes or your liver.
Zollinger-Ellison syndrome may be associated with another disease called multiple endocrine neoplasia type I (MEN I). People with MEN I have multiple tumors in their endocrine system in addition to pancreatic tumors. They also have tumors in their parathyroid glands and may have tumors in their pituitary glands. About 25 percent of people who have gastrinomas have them as part of MEN I.
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| What are the symptoms of the disease? |
| The Danger Signals |
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In many cases of Zollinger-Ellison syndrome, the tumors are noncancerous (benign). However, in half or more of cases, the tumors are cancerous (malignant). If the tumors are cancerous, the danger is that the cancer could spread to your liver and to the lymph nodes near your pancreas and small intestine.
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Burning, aching, gnawing or discomfort in your upper abdomen
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