Zollinger-Ellison Syndrome (ZES) is a very rare condition that causes both tumors and ulcers to form in the stomach and intestine. Tumors may also form on the liver or pancreas. Left untreated, some tumors can metastasize and spread to other parts of the body. ZES is also associated with extreme stomach discomfort. Treatment can reduce discomfort and prevent tumors from spreading.
Researchers know very little about the causes of ZES. However, they do know that ZES causes the gut to produce far too much acid. This in turn leads to the development of ulcers, and usually more than one ulcer exists.
Symptoms of ZES include extreme pain in the upper abdomen. Some patients describe this as a burning or gnawing sensation. Diarrhea or evidence of bleeding by producing black bowel movements is common. Appetite tends to be affected by significant pain, and patients may lose weight quickly. ZES may also cause ongoing nausea or vomiting.
ZES usually starts because a tumor has formed. Tumor location is usually the pancreas or duodenum. Several tumors may be present and are occasionally malignant. Usually the tumors themselves are made up of cells that force the body to produce additional acids, sometimes called gastric juices. The acids then begin to deteriorate the lining of the stomach, creating many small ulcers.
25% of ZES cases occur with a condition called multiple endocrine neoplasia (MEN), which also causes tumors on the pituitary glands. MEN may also result in tumors on the thyroid gland, which can cause a hyperthyroid or hypothyroid state.
Diagnosis of ZES is usually made through complete physical exam, blood tests, and endoscopy. In an endoscopy, a small tube is inserted via the mouth, into the abdomen to look for the presence of ulcers and multiple tumors. Ultrasound may also be used to pinpoint the locations of tumors or ulcers.
Often the tumors and ulcers are located in places difficult to remove, yet removal of the tumors is important to prevent cancer. Once tumors and ulcers are removed, the follow-up treatment consists of medication to block acid from forming. However, tumors frequently recur with this condition, so a return of significant pain in the upper abdomen may mean future surgery.
ZES remains difficult to treat and because it is rare, resulting in only about one in a million cases per year, it is also difficult to study. Unlike the ulcers formed by bacteria, ulcers in ZES cases cannot be treated with antibiotics. Treatment is likely to include at least yearly check-ups, so quality of life may be somewhat affected. However, newer and stronger antacid medications have significantly improved quality of life for those with ZES.
