Zollinger-Ellison Syndrome (ZES) is a rare digestive disorder characterized by the development of one or more tumors, known as gastrinomas, in the pancreas and/or the upper part of the small intestine (duodenum). These gastrinomas produce excessive amounts of the hormone gastrin, leading to an overproduction of stomach acid. This excess acid can result in peptic ulcers in the stomach and intestines, as well as other symptoms such as diarrhea, abdominal pain, and acid reflux [1-3].
Etiology and Risk Factors
The exact cause of ZES is not fully understood. However, it is known that the syndrome begins with the formation of gastrinomas in the pancreas or duodenum, and sometimes in other sites like lymph nodes near the pancreas. These tumors secrete large amounts of gastrin, causing the stomach to produce excessive acid . About 25% of individuals with ZES have it as part of an inherited condition called multiple endocrine neoplasia type 1 (MEN1), which also involves tumors in the parathyroid and pituitary glands . ZES is more common in men, particularly those aged 30 to 50 years .
The symptoms of ZES are similar to those of other ulcers and include:
- Nausea and vomiting
- Weight loss
- Abdominal pain, often burning in nature
- Severe heartburn (GERD)
- Intestinal bleeding, indicated by black or tarry stool or blood in the stool
- Loss of appetite [1-3].
Diagnosis of ZES typically involves measuring stomach acid levels and a blood test to check gastrin levels. Imaging tests may also be ordered to locate tumors .
Treatment for ZES often includes medications such as proton pump inhibitors (PPIs) to manage excess stomach acid and prevent ulcers. These medications include esomeprazole, lansoprazole, pantoprazole, dexlansoprazole, rabeprazole, and omeprazole. In some cases, surgery may be necessary to remove tumors. If tumors have spread to other parts of the body, chemotherapy may be required [1, 3].
Complications and Prognosis
While most gastrinomas grow slowly and do not spread quickly, about half may become cancerous and metastasize. Complications of severe peptic ulcer disease can include gastrointestinal bleeding and scarring, potentially leading to obstruction in the GI tract. The 10-year survival rate for ZES is generally good, but severe disease can occur in some individuals [1, 3].
Zollinger-Ellison Syndrome is a complex condition requiring careful diagnosis and management. Early detection and treatment are crucial for managing symptoms and preventing complications.
- Johns Hopkins Medicine. Zollinger-Ellison Syndrome. Available from: https://www.hopkinsmedicine.org/health/conditions-and-diseases/zollingerellison-syndrome
- Mayo Clinic. Zollinger-Ellison Syndrome. Available from: https://www.mayoclinic.org/diseases-conditions/zollinger-ellison-syndrome/symptoms-causes/syc-20379042
- Cleveland Clinic. Zollinger-Ellison Syndrome. Available from: https://my.clevelandclinic.org/health/diseases/17960-zollinger-ellison-syndrome