Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome is a gastric hypersecretory condition caused by a gastrinoma - a gastrin-producing, non-beta islet cell tumor of the pancreas (but can be extrapancreatic). Excessive gastrin production leads to glandular hyperplasia and hypersecretion of HCl and pepsin, resulting in thickened gastric rugae and multiple peptic ulcers within the stomach, duodenum, and proximal jejunum. The true incidence is unknown, but it has been estimated that it accounts for 0.1 to 1% of peptic ulcers. It may occur at any age, but the initial manifestations are most common between ages 20 and 50.

Cardinal Features:

• Severe and recalcitrant upper gastrointestinal ulcerative disease (90-95% of the patients)
• Gastric hypersecretion refractory to histamine stimulation test
• Hypergastrinemia: >1000 ng/L during while fasting
• Hyperacidity with basal acid output > 15mEq/h
• Diarrhea (30%) and steatorrhea (40%): decreased pH inactivates pancreatic enzymes necessary for fat digestion

Patients with Zollinger-Ellison syndrome are initially managed with proton pump inhibitors and H2 blockers. In those who fail medical management, total gastrectomy and tumor excision, where possible, is indicated.

Radiographic Findings:

• Atypical ulcer location: 20% are gastric

• Multiple ulcers in 10%

• Enlarged rugal folds (differential)

• Decreased peristalsis

The stomach is typically imaged by UGIS and may be followed by CT, which is useful in the diagnosis of the primary tumor and identifying other tumor sites.