Zollinger-Ellison syndrome (ZES) is a rare disorder that leads to tumors on the pancreas. The condition predominantly affects people between 20 and 50, and while it is not fatal, it medication for managing symptoms. Approximately one in one million adults have Zollinger-Ellison syndrome, and men are more likely to develop it than women. In rare instances, the tumors may become cancerous and demand more intensive treatment than medication.
For reasons researchers have yet to understand, ZES causes multiple tumors or gastrinomas to grow in and around the pancreas. Sometimes, these tumors also develop in the first section of the small intestine, the duodenum. Gastrinomas release excessive amounts of a digestive hormone called gastrin. Normally, gastrin is necessary to promote proper digestion of food because it triggers secretion of hydrochloric acid and other gastric juices. However, too much gastrin in the stomach promotes peptic ulcers in the upper intestine and duodenum.
Peptic ulcers have two incarnations. A gastric ulcer is an open sore that develops on the inside of the stomach, while duodenal ulcers grow on the upper part of the intestines. Besides Zollinger-Ellison syndrome, gastrointestinal infections and long-term use of NSAIDs and aspirin can also promote peptic ulcer growth. Unless treated, the ulcers may cause internal bleeding as the sores enlarge or obstruct the digestive tract.
Similar to symptoms of a peptic ulcer, ZES symptoms begin as dull pain or burning sensations between the mid-chest and naval. Discomfort usually happens on an empty stomach, and eating food may or may not relieve pain or burning. Other signs of ZES include:
Symptoms vary among people with ZES. Some report only diarrhea, while others experience a severe acid indigestion condition called GERD or gastroesophageal reflux disorder.
A physician may order tests for ZES if symptoms of a peptic ulcer accompany chronic diarrhea or if the patient has a bleeding ulcer. Blood tests, imaging scans to detect gastrinomas, and an upper GI endoscopy can diagnose Zollinger-Ellison syndrome. Measuring the amount of stomach acid in the stomach can also help doctors reach an accurate diagnosis.
There is currently no evidence to suggest ZES is a genetic disorder. However, people diagnosed with multiple endocrine neoplasia type 1 (MEN 1) may pass these genes onto family members who later discover they have ZES. MEN 1 causes tumors to grow on the parathyroid glands and sometimes on the pituitary gland. About one in four people with gastrinomas have MEN 1.
Surgery to remove one tumor is often successful and helps relieve symptoms by reducing stomach acids. If the individual has multiple tumors or they have spread to the liver, surgery is usually not an option because the tumors are small and difficult to find within the pancreatic tissue.
In some cases, doctors can control tumor growth by destroying tumors through embolization or cutting off the supply of blood. If tumors have reached the liver, chemotherapy may slow tumor growth or remove as much of the tumor as possible.
Drugs called proton pump inhibitors (PPIs) can reduce gastric acid production and alleviate symptoms. By interfering with the ability of certain cells to secrete acid, PPIs can help people with Zollinger-Ellison syndrome avoid surgery. While people over 50 taking PPIs are at an increased risk for spine, wrist, and hip fractures, for most, the risk is minimal and the benefits outweigh the risks.
While this disorder may be cured if tumors are found early and completely removed, the cure rate is less than ten percent. People with ZES can have the disorder for years before doctors find a tumor. Undergoing endoscopic ultrasounds and gastrin blood level testing are the best methods for detecting evidence of this disorder in its early stages.
To reduce symptoms, people with ZES can eat vitamin A-rich foods like spinach, carrots, mangoes, sweet potatoes, and apricots. Vitamin C is also helpful for decreasing stomach acid; strawberries, broccoli, potatoes, and cabbage are rich in this nutrient. Avoid spicy and salty foods, caffeine, and alcohol. Limiting consumption of milk and other dairy products may also help reduce stomach acid.
Symptoms such as severe chest pain, bloody or black stools, rapid weight loss, and signs of malnutrition or dehydration demand immediate medical attention. These symptoms could indicate a life-threatening condition. People with ZES should visit their doctor every six months for a complete check-up and symptom evaluation. Preventive medicine is the best way to live with ZES and stay as healthy as possible.
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