Zollinger-Ellison syndrome

Zollinger-Ellison syndrome (ZES) is a rare condition characterized by the development of one or more gastrin-secreting tumors (gastrinomas) that cause excessive production of gastric acid. These tumors most commonly arise in the duodenum or pancreas and are classified as neuroendocrine tumors. The overproduction of gastrin leads to gastric acid hypersecretion, which results in severe and often refractory peptic ulcers in the stomach, duodenum, and sometimes the jejunum. Patients typically present with abdominal pain, diarrhea, heartburn, and gastrointestinal bleeding. In some cases, the disease can lead to esophageal complications including strictures from chronic acid reflux. Approximately 20–30% of ZES cases occur as part of Multiple Endocrine Neoplasia type 1 (MEN1), an inherited condition caused by mutations in the MEN1 gene. In these familial cases, gastrinomas are associated with tumors of other endocrine glands, including the parathyroid glands and pituitary gland. The remaining 70–80% of cases are sporadic, with no identifiable hereditary component. Gastrinomas in ZES may be malignant, and at the time of diagnosis, a significant proportion of patients already have liver metastases. The primary treatment approach involves high-dose proton pump inhibitors (PPIs) such as omeprazole or lansoprazole to control gastric acid hypersecretion and allow ulcer healing. Surgical resection of the gastrinoma is the only potential curative treatment and is recommended when the tumor is localized and there is no evidence of unresectable metastatic disease. For patients with metastatic gastrinomas, treatment options include somatostatin analogs, chemotherapy, targeted therapies such as everolimus, and peptide receptor radionuclide therapy (PRRT). Long-term monitoring is essential due to the risk of tumor progression and metastasis.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Zollinger-Ellison syndrome