What is Zollinger-Ellison syndrome?
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 the patient's stomach, leading to peptic ulcers.
In Zollinger-Ellison syndrome, tumors called gastrinomas produce large amounts of gastrin. This is a hormone that increases the amount of stomach acid. When a large amount of this substance is produced, too much stomach acid is made. This causes sores in the lining of the digestive tract, called peptic ulcers, to form in the stomach and small intestine. 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 primary tumors are usually located in the pancreas or small intestine. Occasionally they are found in nearby lymph nodes. Very rarely, they can be located in more distant parts of the body such as the ovaries. The tumors may spread to other parts of the body such as the liver, spleen, lymph nodes, bone, skin and lining of the abdominal cavity. The tumors are cancerous in 50 percent of the cases. They 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.
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. 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 the patient's stomach or intestine.