Zollinger-Ellison syndrome is a rare condition in which one or more tumors form in your pancreas or the upper part of your small intestine (duodenum). These tumors, called gastrinomas, secrete large amounts of the hormone gastrin, which causes your stomach to produce too much acid. The excess acid then leads to peptic ulcers, as well as to diarrhea and other symptoms.
Zollinger-Ellison syndrome (ZES) is rare. The disease may occur at any time in life, but people usually find out they're affected between ages 20 and 50. Medications to reduce stomach acid and heal the ulcers are the usual treatment for Zollinger-Ellison syndrome.
Signs and symptoms of Zollinger-Ellison syndrome may include:
- Abdominal pain
- Burning, aching, gnawing or discomfort in your upper abdomen
- Acid reflux and heartburn
- Nausea and vomiting
- Bleeding in your digestive tract
- Unintended weight loss
- Decreased appetite
When to see a doctor
See your doctor if you have a persistent, burning, aching or gnawing pain in your upper abdomen, especially if you've also been experiencing nausea, vomiting and diarrhea.
Tell your doctor if you've used over-the-counter acid-reducing medications such as omeprazole (Prilosec, Zegerid), cimetidine (Tagamet), famotidine (Pepcid) or ranitidine (Zantac) for long periods of time. These medications may mask your symptoms, which could delay your diagnosis.
The exact cause of Zollinger-Ellison syndrome remains unknown. But the sequence of events that occurs in Zollinger-Ellison syndrome is clear. The syndrome begins when a tumor (gastrinoma) or tumors form in your pancreas, duodenum or the lymph nodes adjacent to your pancreas.
Your pancreas sits behind and below your stomach. It produces enzymes that are essential to digesting food. The pancreas also produces several hormones including insulin, a hormone that helps to control your blood glucose.
Digestive juices from the pancreas, liver and gallbladder mix in the duodenum, the part of the small intestine next to your stomach. This is where digestion reaches its peak.
The tumors that occur with Zollinger-Ellison syndrome are made up of cells that secrete large amounts of the hormone gastrin. Increased gastrin makes the stomach produce far too much acid. The excessive acid then leads to peptic ulcers and sometimes to diarrhea.
Besides causing excess acid production, the tumors are cancerous (malignant). Although the tumors tend to grow slowly, the cancer can spread elsewhere — most commonly to nearby lymph nodes or your liver.
Association with MEN 1
Zollinger-Ellison syndrome may be caused by an inherited condition called multiple endocrine neoplasia, type 1 (MEN 1). People with MEN 1 also have tumors in the parathyroid glands and may have tumors in their pituitary glands.
About 25 percent of people who have gastrinomas have them as part of MEN 1. They may also have tumors in the pancreas and other organs.
If you have a blood relative, such as a sibling or parent, with MEN 1, it's more likely that you'll have Zollinger-Ellison syndrome.