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Zollinger-Ellison syndrome


Disease: Zollinger-Ellison syndrome Zollinger-Ellison syndrome
Category: Digestive diseases

Disease Definition:

Zollinger-Ellison syndrome is a complex state in which one or more tumors form in the patient’s pancreas or in the upper part of the patient’s small intestine (duodenum). These tumors secrete large amounts of a hormone which causes huge production of acid by the patient’s stomach. The development of peptic ulcers is the eventual result of the excess acid.


Even though the average age at diagnosis is 50, but the disease could occur at any time. Zollinger-Ellison syndrome is rare.


Usually, medications are used to reduce acid and heal the ulcers. In some cases, surgery to remove the tumors could be an available option.

Work Group:

Prepared by: Scientific Section

Symptoms, Causes


The signs and symptoms of Zollinger-Ellison syndrome are quite similar to those of a peptic ulcer.
These symptoms may be:


  • Nausea and vomiting
  • Burning, aching, gnawing or discomfort in the patient’s upper abdomen
  • Weakness
  • Diarrhea
  • Unintentional weight loss
  • Heartburn
  • Bleeding in the digestive tract


In the case of feeling persistent aching, burning or gnawing pain in the upper abdomen, a person should contact the doctor, particularly if he/she has been experiencing nausea, vomiting and diarrhea.
Without the doctor’s knowledge and advice, The patient shouldn’t use over-the-counter acid-reducing medications such as omeprazole, cimetidine, famotidine or ranitidine for a long time. Those medications might conceal the symptoms of the disease, which will eventually delay diagnosis. Early detection and treatment are vital in the case of Zollinger-Ellison syndrome.


The cause of Zollinger-Ellison syndrome is still not known, however, the sequence of steps of this disease is clear. When a tumor (gastrinoma) or tumors begin to occur in the patient’s pancreas or duodenum, the Zollinger-Ellison syndrome occurs.


The pancreas lies behind and below the stomach, secreting enzymes that are essential to digest food. It also secretes many hormones, such as insulin and glucagon which control the blood sugar levels and the stomach hormone gastrin that regulates production of stomach acid. The upper part of the small intestine called duodenum, starts at the lower end of the stomach. Digestion reaches its peak in the duodenum, where digestive juices from the pancreas, liver and gallbladder are mixed.


In the Zollinger-Ellison syndrome, the tumors that occur consist of cells that produce massive amounts of gastrin, which is a substance that causes the stomach to produce too much acid. Peptic ulcers and sometimes diarrhea could be the result of the excessive amounts of acid.
These tumors could be malignant, in addition to causing excess acid production. Although these tumors grow slowly, but the cancer can spread to other parts of the body, such as the nearby lymph nodes or liver.

MEN 1 (multiple endocrine neoplasia, type 1) is sometimes associated with Zollinger-Ellison syndrome. In addition to pancreatic tumors, people with MEN 1 have multiple tumors in the endocrine system, tumors in the parathyroid glands and in some cases, tumors in the pituitary glands.
Almost 25% of people with gastrinomas have them as part of MEN 1.





In the treatment of Zollinger-Ellison syndrome, the focus is mainly on the treatment of the tumor, and then on the ulcers.



Because the tumors that occur in Zollinger-Ellison syndrome are generally small and very difficult to locate, great skills are required in removing them.


Although the tumor may be surgically removed if there’s only one, but if someone has multiple tumors or tumors that have spread to the liver, surgery may not be an available option. In some cases, when the tumors are multiple, removal of a single large tumor may be recommended.


Sometimes doctors suggest other options to control tumor growth, such as :


  • Trying to destroy the tumor by using heat to destroy cancer cells (radiofrequency ablation) or by cutting off the blood supply (embolization).
  • Liver transplant
  • Using chemotherapy in an attempt to slow the growth of the tumor.
  • Removing as much as possible of a liver tumor (debulking).
  • Injecting drugs into the tumor to relieve cancer symptoms.


Severing the nerves that promote acid secretion or removing the entire stomach, which are more radical surgical approaches, aren’t generally done these days because medications are successful in controlling ulcers and acid production.



Almost always excess acid production can be controlled nowadays. The first line of treatment is the medications known as proton pump inhibitors. In Zollinger-Ellison syndrome, these are the most effective medications for decreasing acid secretion. Proton pump inhibitors are very effective drugs, by preventing the action of the tiny “pumps” within acid-secreting cells, they reduce the amount of acid. The most prescribed medications include lansoprazole (omeprazole), rabeprazole, pantaprazole and esomeprazole magnesium.


Although in the past histamine (H-2) blockers were used in treating the ulcers of Zollinger-Ellison syndrome, however, they aren’t as effective as the proton pump inhibitors.


One of several surgeries may be recommended to treat peptic ulcers .This may include surgery to :


  • Close up the hole that an ulcer has made in the wall of the duodenum or the stomach
  • Stop an ulcer from bleeding
  • Relieve an obstruction caused by an ulcer


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