Overview

Diagnosis

Zollinger-Ellison syndrome (ZES) is diagnosed through a combination of clinical evaluation, laboratory tests, and imaging studies. The goal of diagnosis is to detect the presence of gastrin-producing tumors, known as gastrinomas, and confirm the excessive production of stomach acid.

Common diagnostic steps include:

  • Blood tests to measure gastrin levels. Elevated fasting gastrin levels strongly suggest ZES, especially when stomach acid production is also high.

  • Secretin stimulation test to confirm the diagnosis. In this test, a hormone called secretin is given, and gastrin levels are measured afterward. In people with ZES, gastrin levels increase abnormally.

  • Stomach acid measurement to check if the stomach is producing too much acid, which supports the diagnosis.

  • Imaging tests such as CT scan, MRI, or endoscopic ultrasound to locate the gastrinomas. These tests can identify tumors in the pancreas, duodenum, or surrounding tissues.

  • Somatostatin receptor scintigraphy (OctreoScan) or PET scans to detect small tumors or metastases that may not appear on other imaging tests.

Because ZES can occur as part of a genetic disorder called multiple endocrine neoplasia type 1 (MEN1), genetic testing may also be recommended for some patients.

Treatment

Treatment for Zollinger-Ellison syndrome focuses on controlling stomach acid production and managing gastrinomas. The approach depends on whether the tumors are cancerous and whether they have spread.

Common treatment options include:

  • Proton pump inhibitors (PPIs): These medications, such as omeprazole or pantoprazole, are highly effective in reducing stomach acid production and preventing ulcers.

  • Surgery: If tumors are localized and operable, surgery may be performed to remove gastrinomas completely. This can potentially cure the condition.

  • Somatostatin analogs: Drugs like octreotide may help control both acid production and tumor growth.

  • Chemotherapy or targeted therapy: Used in cases where tumors are malignant or have spread to other organs.

  • Management of MEN1: If ZES is part of MEN1, regular monitoring and treatment of other hormone-related tumors are important.

Complications

If left untreated, Zollinger-Ellison syndrome can lead to serious complications such as:

  • Peptic ulcers that may bleed, perforate, or cause intestinal obstruction

  • Severe acid reflux and esophagitis

  • Chronic diarrhea due to high acid levels affecting digestion

  • Tumor spread (metastasis), especially to the liver or lymph nodes

Effective treatment and regular follow-up can help prevent most of these complications and improve quality of life.

Outlook

With modern treatments, most people with Zollinger-Ellison syndrome can manage symptoms effectively and live normal lives. However, long-term medical care is essential, especially if gastrinomas are malignant or associated with MEN1.

Early diagnosis, acid suppression therapy, and timely surgical intervention offer the best outcomes for individuals with this rare but treatable condition.

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