Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome

Zollinger-Ellison syndrome is a disorder where increased levels of the hormone gastrin are produced, causing the stomach to produce excess hydrochloric acid. Often, the cause is a tumor of the pancreas producing the hormone gastrin.

Zollinger-Ellison syndrome is caused by tumors usually found in the head of the pancreas and the upper small bowel (over 90% in the so called gastrinoma triangle, bounded by the porta hepaticus, the neck of the pancreas, and the 2nd part of the duodenum). These tumors produce the hormone gastrin and are called gastrinomas. High levels of gastrin cause overproduction of stomach acid.
Gastrin works on stomach parietal cells causing them to secrete more hydrogen ions into the stomach lumen. In addition, gastrin acts as a trophic factor for parietal cells, causing parietal cell hyperplasia. Thus, there is an increase in the number of acid secreting cells and each of these cells produces acid at a higher rate. The increase in acidity contributes to the development of peptic ulcers in the stomach and duodenum. High acid levels lead to multiple ulcers in the stomach and small bowel.

Patients with Zollinger-Ellison syndrome may experience abdominal pain and diarrhea. The diagnosis is also suspected in patients without symptoms who have severe ulceration of the stomach and small bowel.

Gastrinomas may occur as single tumors or as multiple, small tumors. About one-half to two-thirds of single gastrinomas are malignant tumors that most commonly spread to the liver and lymph nodes near the pancreas and small bowel. Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). MEN I patients have tumors in their pituitary gland and parathyroid glands in addition to tumors of the pancreas.

Epigastric pain (stomachache) that goes away with consumption of foods
Vomiting blood (hematemesis) (occasional)
Difficulty in eating

Proton pump inhibitors and H2 blockers are used to slow down acid secretion. If possible the tumours should be surgically removed, or treated with chemotherapy.

The disease entity was first described in 1955 by its namesakes: Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg 1955;142:709-23. PMID 13259432.

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