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Zollinger-Ellison Syndrome.

Patrick D. Hung¹, Mitchell L. Schubert, Anastasios A. Mihas

¹McGuire Veterans Administration Medical Center, Division of Gastroenterology, 111N, 1201 Broad Rock Boulevard, Richmond, VA 23249, USA. mitchell.schubert@med.va.gov

Current Treatment Options in Gastroenterology

|March 12, 2003

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Zollinger-Ellison syndrome (ZES) is a condition caused by gastrinomas, leading to excess stomach acid. Early diagnosis involves serum gastrin levels and imaging, with proton pump inhibitors as the primary treatment.

Area of Science:

Gastroenterology
Endocrinology
Oncology

Background:

Zollinger-Ellison syndrome (ZES) results from gastrinomas, tumors that cause excessive gastric acid secretion.
Gastrin stimulates acid production and leads to hyperplasia of parietal and enterochromaffin-like (ECL) cells.
Suspicion of ZES is warranted in patients with severe esophagitis, multiple or refractory peptic ulcers, ulcers in unusual locations, diarrhea, or a family history of Multiple Endocrine Neoplasia type 1 (MEN-1).

Purpose of the Study:

To outline the diagnostic criteria and management strategies for Zollinger-Ellison syndrome.
To highlight the importance of early detection and appropriate treatment for ZES patients.
To review the role of various diagnostic modalities and therapeutic interventions for gastrinomas.

Main Methods:

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Initial diagnosis involves measuring fasting serum gastrin levels after discontinuing antisecretory medications.
Gastric acidity is assessed via pH or gastric analysis if gastrin levels are elevated.
Secretin stimulation tests can aid in diagnosing ZES with mild hypergastrinemia.
Localization studies include somatostatin receptor scintigraphy (SRS) and endoscopic ultrasound (EUS).

Main Results:

High-dose oral proton pump inhibitors (PPIs) are the first-line treatment for ZES.
Parenteral pantoprazole is recommended for cases requiring intravenous therapy.
SRS and EUS combined detect over 90% of gastrinomas.
Surgical cure is achievable in 30% of patients without metastasis and MEN-1.
Surgical resection is advised for gastrinomas larger than 2.5 cm to reduce metastasis risk.

Conclusions:

ZES diagnosis relies on elevated gastrin levels and confirmed gastric acid hypersecretion.
Effective management involves high-dose PPIs, with surgery reserved for specific cases.
Advanced imaging techniques like SRS and EUS are crucial for tumor localization.
Surgical intervention offers a potential cure in select patients and can mitigate metastatic risk.