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Zollinger-Ellison Syndrome


Zollinger-Ellison syndrome is a rare digestive disorder that results in excessive formation of the hormone gastrin. This hormone is released from our stomach normally to aid in digestion. Excessive production of gastrin can increase the release of gastric acid from your stomach, which in turn can cause ulceration of the digestive tract. In this condition, one or more tumors form in your pancreas or the initial part of your small intestine. These tumors, called gastrinomas, produce accessory gastrin. Certain medications and treatment options can manage Zollinger-Ellison syndrome. 


The exact cause of Zollinger-Ellison syndrome is unknown. Like most tumors, the cause is difficult to identify. Only the mechanism of this disease is understood. In Zollinger-Ellison syndrome, tumors that form on the pancreas and other parts of the digestive tract secrete the hormone gastrin (hence called gastrinomas). This hormone triggers the release of gastric acid from your stomach. A large amount of gastric acid disturbs the pH of your stomach and small intestines, leading to digestive issues. Acid also erodes the epithelial lining of the digestive tract, which leads to ulcers, predominantly in the stomach and duodenum (the initial part of the small intestine). These can be painful and uncomfortable for the affected person.


According to research, Zollinger-Ellison syndrome has also been linked with Multiple Endocrine Neoplasia type 1 (MEN 1). In this condition, tumors are formed in multiple parts of the body, including the pituitary gland, parathyroid gland, and pancreas. MEN 1 is an inherited disease. Almost 25 to 30% of people with Zollinger-Ellison syndrome have it due to MEN 1. 

Risk Factors And Epidemiology

Because the exact cause of this condition has not been identified, it is difficult to state the risk factors associated with it. However, the link of Zollinger-Ellison syndrome with MEN 1 should be considered. If you have a family history of MEN 1, it is possible that you may develop Zollinger-Ellison syndrome at some point in your life. Alcohol abuse has also been noticed as a risk factor for this condition, but the exact effects of alcohol on the development of this condition are unclear.


Zollinger-Ellison syndrome can affect people of any age, but it is frequently observed in individuals aged 20 to 50 years. Males are more commonly affected by this disease than females. There is no racial predominance for this condition. 

Signs And Symptoms

Two of the most common symptoms of Zollinger-Ellison syndrome are abdominal pain and chronic diarrhea. Majority of its symptoms resemble that of digestive tract ulcers which include acid reflux, burning sensation in the chest region, regurgitation, burping, nausea/ vomiting, loss of appetite, and unintended weight loss. If the disease persists for a long time and the ulcers proliferate, you may develop signs of intestinal bleeding. This includes dark-colored stools and presence of blood in the stool.   


The diagnosis of Zollinger-Ellison syndrome begins with obtaining the history of your symptoms and family history. A physical examination may be done in some cases. The initial laboratory testing involves testing your blood samples to look for increased gastrin levels. This test is not confirmatory because elevated gastrin levels can also occur because of other conditions. Another test is done, which is called the secretin stimulation test. If this test comes back positive, you likely have Zollinger-Ellison syndrome. Endoscopy of your upper intestinal tract is also an effective test to visualize ulcers caused by this condition. Your doctor may also perform an endoscopic ultrasound to find out the location of tumors. Other imaging tests such as CT scans and MRI can also help identify the position and size of gastrinomas. 

Differential Diagnosis

Zollinger-Ellison syndrome should be differentiated from other conditions that cause abdominal pain and diarrhea. Examples of such conditions include peptic ulcers, gastroesophageal reflux disease, hypergastrinemia, gastric antrum syndrome, Crohn’s disease, celiac disease, irritable bowel syndrome, etc. 


Treatment of Zollinger-Ellison syndrome is based on the severity of the condition. If you have only one tumor, it can be excised via surgery. Often these tumors are small in size and difficult to locate. If you have multiple tumors, your doctor may suggest other treatment options, including debulking, embolization, radiofrequency ablation, injection of drugs directly into the tumor, or chemotherapy. A liver transplant will be necessary in severe cases where the tumor has metastasized to the liver and isn’t treatable with surgical options. The second part of treatment involves treating the ulcers. This can be done by taking medications to decrease gastric acid production. 


Medications given for Zollinger-Ellison syndrome control gastric acid release and prevent its effects on the lining of the digestive tract. Common medications given for this disease are proton pump inhibitors that reduce gastric acid production. These include omeprazole, lansoprazole, esomeprazole, rabeprazole etc. These medications should only be taken in limited dosages as prescribed by your doctor. Long-term use without dosage control can lead to other complications. 


The prognosis of Zollinger-Ellison syndrome is good when it is treated with medical and surgical measures. The risk of complications is higher if it is left untreated. It can lead to anemia, perforated ulcers, weight loss, and eventual death. 


There are no determined methods to prevent Zollinger-Ellison syndrome because the cause of this disease is still unidentified. The only precaution taken is genetic testing if you have a family history of MEN 1. However, it is still difficult to predict the possibility of developing this condition even if you have a positive family history. 

Our clinical experts continually monitor the health and medical content posted on CURA4U, and we update our blogs and articles when new information becomes available. Last reviewed by Dr.Saad Zia on May 09, 2023.





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