What is the definition of Zollinger-Ellison Syndrome?

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.

What are the alternative names for Zollinger-Ellison Syndrome?

Z-E syndrome; Gastrinoma

What are the causes for Zollinger-Ellison Syndrome?

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.

What are the symptoms for Zollinger-Ellison Syndrome?

Symptoms may include:

  • Abdominal pain
  • Diarrhea
  • Vomiting blood (sometimes)
  • Severe esophageal reflux (GERD) symptoms

Signs include ulcers in the stomach and small intestine.

What are the current treatments for Zollinger-Ellison Syndrome?

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.

What is the outlook (prognosis) for Zollinger-Ellison Syndrome?

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.

What are the possible complications for Zollinger-Ellison Syndrome?

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 should I contact a medical professional for Zollinger-Ellison Syndrome?

Contact your health care provider if you have severe abdominal pain that does not go away, especially if it occurs with diarrhea.

Endocrine

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.

  • Condition: Neuroendocrine Neoplasms (NENs) of the Digestive Tract
  • Journal: Nutrients
  • Treatment Used: Surgery, Somatostatin Analogue Therapy, Radionuclide Therapy, Chemotherapy, Molecular Targeted Therapies, Alpha-Interferon Therapy, and Inhibitors of Serotonin Production
  • Number of Patients: 0
  • Published —
This article discusses neuroendocrine neoplasms (NENs) of the digestive tract, their treatment, and nutritional therapy.
  • Condition: Hyper-Gastrinemia and Gastric Carcinoids
  • Journal: International journal of molecular sciences
  • Treatment Used: Proton Pump Inhibitors (PPIs)
  • Number of Patients: 0
  • Published —
This article discusses the relationships between hypergastrinemia, gastric carcinoids, and the use of proton pump inhibitors (PPIs).