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.
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 rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
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.
Symptoms
Symptoms may include:
- Abdominal pain
- Diarrhea
- Vomiting blood (sometimes)
- Severe esophageal reflux (GERD) symptoms
Signs include ulcers in the stomach and small intestine.
Exams and Tests
Tests include:
- Abdominal CT scan
- Calcium infusion test
- Endoscopic ultrasound
- Exploratory surgery
- Gastrin blood level
- Octreotide scan
- Secretin stimulation test
Treatment
Drugs called proton pump inhibitors (omeprazole, lansoprazole, and others) are 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 is 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
- Intestinal bleeding or hole (perforation) from ulcers in the stomach or duodenum
- Severe diarrhea and weight loss
- Spread of the tumor to other organs
When to Contact a Medical Professional
Contact your health care provider if you have severe abdominal pain that does not go away, especially if it occurs with diarrhea.
References
Öberg K. Neuroendocrine tumors and related disorders. In: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Textbook of Endocrinology. 14th ed. Philadelphia, PA: Elsevier; 2020:chap 45.
Strosberg JR, Al-Toubah T. Neuroendocrine tumors. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 34.
Version Info
Last reviewed on: 11/2/2022
Reviewed by: Michael M. Phillips, MD, Emeritus Professor of Medicine, The George Washington University School of Medicine, Washington, DC. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
