Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome is a condition in which small tumors, called gastrinomas, are formed in the pancreas or duodendum. The tumors secrete a substance known as gastrin that results in excessive acid secretion in the stomach.

As a result, between 90 and 95 percent of persons with Zollinger-Ellison syndrome have peptic ulcers at some point during their disease. Ulcers associated with Zollinger-Ellison syndrome are typically more persistent and respond less well to treatment than do typical peptic ulcers. It is estimated that Zollinger-Ellison syndrome is responsible for up to 1 percent of all peptic ulcers. Zollinger-Ellison syndrome may appear at any age, however the symptoms are most likely to make an appearance after the age of 30 and before the age of 60.

If you have the symptoms of Zollinger-Ellison syndrome, your physician will perform blood tests to see whether the gastrin level in your blood is increased. Generally, a person with Zollinger-Ellison syndrome has an abnormally high serum gastrin level in addition to producing excessive amounts of acid.

Your physician may also order a barium x-ray of your stomach, duodenum, and sometimes, jejunum or upper gastrointestinal endoscopy to detect the presence of ulcers. Because these gastrinomas are often very small and difficult to locate, treatment preciously was directed at solving the ulcer disease rather than dealing directly with the tumor.

However, recent advances in high-quality ultrasound that can be used in the operating room and nuclear medicine scans that use a tiny dose of radioactive material that attaches to the tumor and shows as a hot spot on the scan have increased the successful identification of these tumors.

Zollinger-Ellison syndrome may be a serious disease. In half to two-thirds of the patients, the gastrinomas are malignant and slowly spread, most commonly to the lymph nodes and liver. Moreover, the ulcer disease associated with this syndrome usually is sever and not easily treated with conventional ulcer medication or surgery. As with most serious diseases, the earlier the condition is diagnosed, the better the chances of a favorable outcome.

Typically, the ulcer disease in Zollinger-Ellison syndrome is resistant to standard doses of ulcer medication and conventional surgery. Especially if you are young, your physician may elect to perform an operation to identify and remove the tumor responsible for the excess production of acid. Sometimes these tumors are small or multiple in nature and therefore cannot be located or removed.

If your surgeon is unable to remove the gastrinoma, you will need to take an acid-reducing medication. The most effective drug is omeprazole, which has the advantage of virtually eliminating acid production for a period of time with small doses.