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Zollinger-Ellison Syndrome – Symptoms, Treatment

Zollinger-Ellison syndrome is a serious condition that is characterized by the development of a group of tumors in the pancreas or on the lymph nodes present near the pancreas. The pancreas is an organ situated under and behind the stomach and above the upper part of the small intestine.

The Zollinger-Ellison syndrome tumors are also called gastrinomas. The presence of such tumors causes the excessive production of the hormone gastrin, which in turn results in increased manufacture of acids in the stomach. The excessive quantities of acid in the stomach eventually facilitate the development of peptic ulcers.

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Zollinger-Ellison syndrome generally tends to affect individuals between the ages of 20 to 50 years and is more common in men than women.

Different types of medications are used to treat the peptic ulcers and to reduce the excess stomach acids that accompany Zollinger-Ellison syndrome. The tumors affecting the pancreas can be removed via surgical methods or other methods recommended by the doctors.

Symptoms of Zollinger-Ellison syndrome

The signs and symptoms of Zollinger-Ellison syndrome are almost similar to the ones experienced with peptic ulcers. Some of the symptoms are as follows:

  • The upper stomach may experience discomfort, aching and burning sensations
  • Diarrhea
  • Heartburn
  • Abdominal pain
  • Anemia
  • Nausea and/or vomiting
  • Lack or loss of appetite
  • An overall weakness or weariness
  • There may be bleeding in digestive tract
  • Inexplicable loss in weight

It is essential for affected individuals to immediately consult a doctor for diagnosis and treatment, when they experience the above mentioned symptoms.

It is important to note that prolonged use of acid control drugs can result in lack of symptoms, even when affected by Zollinger-Ellison syndrome. Hence for correct diagnosis, it is important for the affected patients to inform the doctor about the intake of such over the counter medications.

Causes of Zollinger-Ellison syndrome

The exact cause of Zollinger-Ellison syndrome is as yet unknown. However, doctors are aware of a number of causes that can eventually result in the formation of the painful condition

Zollinger-Ellison syndrome generally begins with the development of tumors in the pancreas, the duodenum and/or in the lymph nodes that are present near the pancreas. The pancreas is responsible for the production of many hormones. The hormone gastrin is one such hormone and it regulates acid production in the stomach.

The duodenum refers to the upper region of the small intestine. The digestives juices released by the gallbladder, pancreas and the liver get mixed in the duodenum. It is the most important part of the digestive process.

The tumors associated with Zollinger-Ellison syndrome release excessive amounts of the hormone gastrin, which encourages the stomach to make increased amounts of acid. The acids result in the formation of ulcers and can also sometimes lead to diarrhea.

Association of Zollinger-Ellison syndrome with MEN 1

A few studies suggest that Zollinger-Ellison syndrome can also result from a hereditary disorder known as multiple endocrine neoplasia Type 1 or MEN 1. In this case, the affected individuals tend to develop several tumors in the endocrine system, in addition to the tumors that occur in the pancreas. The tumors may also be present in the pituitary glands and the parathyroid glands. Around 25 percent of individuals who develop the Zollinger-Ellison syndrome tumors, also experience the above symptoms, as a part of MEN 1

Zollinger-Ellison syndrome treatment

The initial treatment for Zollinger-Ellison syndrome involves medications for managing the ulcers and removal of the tumors. Once the tumors responsible for the ulcers are successfully treated/removed, then the ulcers medications are discontinued.

Tumor treatment: The Zollinger-Ellison syndrome tumors are generally very tiny and thus difficult to locate. Hence, experienced surgeons are needed to locate and remove such tumors. The presence of several such tumors or the spread of these tumors to other organs like the liver can pose many problems in their complete and effective removal. In such uncommon cases, the following additional treatment methods may be adopted:

  • Removal of all the tumors that can be found and thus debulking the liver
  • Destruction of the tumor cells via a treatment method known as radiofrequency ablation
  • A Liver transplant
  • Blocking the supply of blood to the tumors via certain medications and thus destroying the tumors
  • Chemotherapy that involves the use of potent drugs that are designed to specifically destroy the tumor cells.

Excess acid and ulcer treatment: The ulcers and increased acid production caused by Zollinger-Ellison syndrome are generally treated by medications. Surgical treatment methods such as cutting the nerves that induce the stomach to make more acid, or partial or complete removal of the stomach are generally not recommended. The use of drugs such as proton pump inhibitors is usually considered as the best way to control the increased acid production. The drug is quite potent and works by blocking the tiny outlets in acid producing cells, thereby reducing the acid levels.

Surgery for Zollinger-Ellison syndrome peptic ulcers may be required to correct the following abnormalities:

  • To stop bleeding of the ulcers
  • To close any holes in the stomach or duodenum caused by the ulcers
  • To remove any blockages caused by the ulcers
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