ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences
Zollinger-Ellison syndrome
Print
Other Resources UpToDate PubMed

Zollinger-Ellison syndrome

Print Images (1)
Contributors: Justin Graf, Christine Osborne MD, Jennifer J. Findeis-Hosey MD
Other Resources UpToDate PubMed

Synopsis

Zollinger-Ellison Syndrome (ZES) is severe peptic ulcer disease secondary to gastric acid hypersecretion due to unregulated gastrin release from a gastrinoma. Elevated gastrin leads to overstimulation of histamine-secreting enterochromaffin-like cells and subsequent stimulation of parietal cell production of hydrochloric acid. Long-standing hypersecretion of gastric acid leads to peptic ulcer disease, erosive esophagitis, and diarrhea.

The incidence of ZES ranges from 0.1%-1% of patients with peptic ulcer disease and is more common in males than in females. Most patients are diagnosed between ages 20-50, although the time span from symptoms to diagnosis is typically greater than 5 years due to the wide availability of acid-suppressing medications. 70%-80% of gastrinomas are sporadic, and 20%-30% are secondary to multiple endocrine neoplasia type 1 (MEN1). Gastrinomas typically arise in the duodenum and are one of the most common functional pancreatic neuroendocrine tumors. In contrast with pancreatic gastrinomas, duodenal gastrinomas are generally small (<1 cm), often multiple, and more likely to have metastasized to the liver at the time of diagnosis. In a small percentage of patients, gastrinomas arise in a nonpancreatic or nonduodenal site, including the stomach and ovary.

Abdominal pain and chronic diarrhea are the most the most common presenting symptoms of ZES, each seen in approximately 75% of patients. Other common symptoms include heartburn, weight loss, and gastrointestinal bleeding.

Codes

ICD10CM:
E16.4 – Increased secretion of gastrin

SNOMEDCT:
53132006 – Zollinger-Ellison syndrome

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

  • Antral G-cell hyperplasia – Characterized by poor response to secretin stimulating test and absence of gastrinoma on imaging.
  • Retained antrum syndrome – Presents as recurrent peptic ulcers following gastrectomy. Gastrin elevation is modest compared to ZES.
  • Sporadic peptic ulcer disease
  • Celiac disease
  • Gastric malignancy
  • Chronic pancreatitis
  • Atrophic gastritis
  • Vagotomy
  • Acid-suppressive medications

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed: 05/08/2018
Last Updated: 06/18/2018
Copyright © 2019 VisualDx®. All rights reserved.
Zollinger-Ellison syndrome
Print 1 Images
Zollinger-Ellison syndrome : Abdominal pain, Diarrhea, Epigastric pain, Melena, Dyspepsia
Copyright © 2019 VisualDx®. All rights reserved.