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Understanding Zollinger-Ellison Syndrome: Symptoms, Diagnosis, and Treatment

Jerome Albert Ecker | MD

Medically reviewed by Jerome Albert Ecker | MD, Assistant Professor of Medicine, Duke University - Durham, NC on August 27th, 2023.

Key Takeaways

  • ZES is caused by gastrin-secreting tumors (gastrinomas) that stimulate massive stomach acid production, leading to severe peptic ulcers in unusual locations like the jejunum and recurrent ulcers despite standard treatment.

  • 60-90% of gastrinomas occur in the "gastrinoma triangle" bordered by the cystic duct, pancreatic neck, and duodenum; 50-60% are malignant with potential for liver metastases.

  • Symptoms include severe abdominal pain, diarrhea (due to acid inactivating digestive enzymes), and complications from aggressive ulcers like bleeding, perforation, or gastric outlet obstruction.

  • Diagnosis requires fasting serum gastrin levels (typically >1000 pg/mL, normal <100) combined with gastric pH <2, confirmed with secretin stimulation test showing paradoxical gastrin rise.

  • 25% of ZES cases occur within Multiple Endocrine Neoplasia type 1 (MEN1)—these patients need screening for parathyroid and pituitary tumors and have different surgical approaches due to multiple small tumors.

Zollinger-Ellison Syndrome (ZES) is a rare condition that affects the gastrointestinal system. People with ZES develop tumors called gastrinomas in their pancreas and duodenum (the first part of the small intestine). These tumors secrete a hormone called gastrin, which causes the stomach to produce excessive amounts of acid, leading to the development of stomach and duodenal ulcers in 90% of ZES patients.

Symptoms of Zollinger-Ellison Syndrome

Not everyone with ZES experiences symptoms, but when they do occur, they may include:

  • Abdominal pain and burning sensation

  • Nausea and vomiting

  • Diarrhea

  • Weight loss

  • Stomach bleeding

  • Weakness and fatigue

Diagnosing Zollinger-Ellison Syndrome

If your doctor suspects you have ZES, they will perform several tests to confirm the diagnosis. These may include:

  1. Blood tests to check for high levels of gastrin

  2. Tests to measure stomach acid production

  3. Endoscopy with ultrasound to examine the esophagus, stomach, and duodenum and locate tumors

  4. CT scan, PET scan, or octreotide scan to identify gastrinomas and neuroendocrine (hormone-producing) tumor cells

Despite these tests, gastrinomas can be challenging to locate.

Understanding Zollinger-Ellison Syndrome: Symptoms, Diagnosis, and Treatment

Treatment Options for Zollinger-Ellison Syndrome

Treatment for ZES focuses on reducing stomach acid production and addressing the underlying gastrinomas. Proton pump inhibitors (PPIs) such as omeprazole, lansoprazole, and pantoprazole are commonly prescribed to curb acid production and allow ulcers to heal. Somatostatin analogs like octreotide can also help control symptoms by suppressing hormone production.

The approach to treating gastrinomas depends on whether they are sporadic or part of the inherited MEN I syndrome. Sporadic gastrinomas are typically treated with acid suppression and surgical removal of the tumor, while MEN I-related gastrinomas are usually managed with acid suppression alone. In cases of metastatic disease, a combination of surgery, chemotherapy, targeted drug therapy, or radiation may be recommended.

Prognosis and Follow-up for Zollinger-Ellison Syndrome

The outlook for people with ZES depends on whether the gastrinomas are cancerous and if they have spread. The five-year survival rate may be 90% if the tumors have not spread to the liver. Surgery can completely cure 20-25% of patients if the gastrinoma is successfully removed. Regular follow-up with your doctor is essential to monitor for recurrence of gastrinomas.

For more information on Zollinger-Ellison Syndrome, visit the following reputable sources:

The Bottom Line

High-dose proton pump inhibitors effectively control acid secretion, while surgical resection offers potential cure for localized sporadic gastrinomas. If you have recurrent or unusually located ulcers, Doctronic can help evaluate whether further workup for ZES is warranted.

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