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Zollinger-Ellison syndrome
Z-E syndrome; Gastrinoma
Zollinger-Ellison syndrome is a condition in which the body produces too much of the hormone gastrin. Most of the time, a small tumor (gastrinoma) in the pancreas or small intestine is the source of the extra gastrin in the blood.
Causes
Zollinger-Ellison syndrome is caused by tumors. These growths are most often found in the head of the pancreas and the upper small intestine. The tumors are called gastrinomas. High levels of gastrin cause production of too much stomach acid.
Gastrinomas occur as single tumors or several tumors. One-half to two-thirds of single gastrinomas are cancerous (malignant) tumors. These tumors often spread to the liver and nearby lymph nodes.
Many people with gastrinomas have several tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). Tumors may develop in the pituitary gland (brain) and parathyroid gland (neck) as well as in the pancreas.
Multiple endocrine neoplasia type I
Multiple endocrine neoplasia (MEN) type I is a disease in which one or more of the endocrine glands are overactive or forms a tumor. It is passed do...
Symptoms
Symptoms may include:
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Abdominal pain
Abdominal pain
Abdominal pain is pain that you feel anywhere between your chest and groin. This is often referred to as the stomach region or belly.
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Vomiting blood
(sometimes)
Vomiting blood
Vomiting blood is regurgitating (throwing up) contents of the stomach that contains blood. Vomited blood may appear either a bright red or dark red c...
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Exams and Tests
Signs include ulcers in the stomach and small intestine.
Tests include:
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Abdominal CT scan
Abdominal CT scan
An abdominal CT scan is an imaging method. This test uses x-rays to create cross-sectional pictures of the belly area. CT stands for computed tomog...
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Endoscopic
ultrasound
Endoscopic
Endoscopy is a way of looking inside the body using a flexible tube that has a small camera and light on the end of it. This instrument is called an...
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Gastrin blood level
Gastrin blood level
The gastrin blood test measures the amount of the hormone gastrin in blood.
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Secretin stimulation test
Secretin stimulation test
The secretin stimulation test measures the ability of the pancreas to respond to a hormone called secretin. The small intestine produces secretin wh...
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Treatment
Drugs called proton pump inhibitors (omeprazole, lansoprazole, and others) used for treating this problem. These drugs reduce acid production by the stomach. This helps the ulcers in the stomach and small intestine heal. These medicines also relieve abdominal pain and diarrhea.
Surgery to remove a single gastrinoma may be done if the tumors have not spread to other organs. Surgery on the stomach (gastrectomy) to control acid production is rarely needed.
Outlook (Prognosis)
The cure rate is low even when it is found early and the tumor removed. However, gastrinomas grow slowly. People with this condition may live for many years after the tumor is found. Acid-suppressing medicines work well to control the symptoms.
Possible Complications
Complications may include:
- Failure to locate the tumor during surgery
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Intestinal bleeding or hole (perforation) from ulcers in the stomach or
duodenum
Duodenum
The duodenum is the first part of the small intestine. It is located between the stomach and the middle part of the small intestine, or jejunum. Aft...
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Severe diarrhea and
weight loss
Weight loss
Unexplained weight loss is a decrease in body weight, when you did not try to lose the weight on your own. Many people gain and lose weight. Uninten...
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When to Contact a Medical Professional
Call your health care provider if you have severe abdominal pain that does not go away, especially if it occurs with diarrhea.
References
Jensen RT, Norton JA. Endocrine tumors of the pancreas and gastrointestinal tract. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease . 9th ed. Philadelphia, PA: Elsevier Saunders; 2010:chap 32.
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Endocrine glands - illustration
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Endocrine glands
illustration
-
Endocrine glands - illustration
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Endocrine glands
illustration
Review Date: 11/20/2014
Reviewed By: Jenifer K. Lehrer, MD, Department of Gastroenterology, Frankford-Torresdale Hospital, Aria Health System, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
