Zollinger-Ellison Syndrome | Digestive Health | Loyola Medicine

Zollinger-Ellison Syndrome

Overview and Facts about Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome is a rare condition caused by tumors in the pancreas or the duodenum, the part of the small intestine that connects to the stomach. The tumors associated with Zollinger-Ellison syndrome are called gastrinomas, which secrete large amounts of the hormone gastrin.

Gastrin causes your stomach to produce acid as part of the normal digestive process; excess gastrin may cause complications affecting your digestive health.

Gastrinomas are slow-growing and may form as a single tumor or as multiple small tumors. These tumors may be cancerous (malignant) and could spread to other parts of the body, especially the liver or nearby lymph nodes.

Causes and Risk Factors of Zollinger-Ellison Syndrome

What actually causes Zollinger-Ellison syndrome is largely unknown. However, the syndrome develops when a specific type of tumor forms in the pancreas or duodenum.

The tumor, called a gastrinoma, is made of cells that secrete a large amount of the hormone gastrin. In turn, gastrin causes the stomach to produce excess acid, which can lead to ulcers and other digestive health problems.

Risk factors for developing Zollinger-Ellison syndrome include having a sibling or parent with an inherited condition called multiple endocrine neoplasia, type 1.

This condition may be responsible for the growth of tumors in the pancreas and other organs involved in hormone production.

Signs and Symptoms of Zollinger-Ellison Syndrome

Signs and symptoms of Zollinger-Ellison syndrome typically affect adults between 20 and 50 years old. Certain over-the-counter medications used to reduce acid production may mask initial symptoms, however, and delay diagnosis.

Signs and symptoms of Zollinger-Ellison syndrome include:

  • Abdominal pain
  • Burning, aching or discomfort in your abdomen
  • Diarrhea
  • Loss of appetite
  • Nausea
  • Ulcers in the stomach or small intestine
  • Vomiting blood
  • Weight loss

Tests and Diagnosis of Zollinger-Ellison Syndrome

To diagnose Zollinger-Ellison syndrome, your doctor will discuss your health and medical history. They may also take the following actions:

  • Performing an endoscopy, which involves inserting a flexible tube with a tiny camera on the end down your throat and into your stomach to look for ulcers.
  • Running a blood test to analyze whether your body is producing elevated levels of gastrin.
  • Taking a biopsy, or tissue sample, to test for gastrin-producing tumor cells.
  • Using imaging tests like ultrasound, MRI and CT scans to locate the tumors.

Treatment and Care for Zollinger-Ellison Syndrome

Treatment of Zollinger-Ellison syndrome includes treating both the tumors and the ulcers caused by them. While surgery may be performed to remove a single tumor, gastrinomas are often small and can spread to your liver, making this option difficult.

Chemotherapy or radiofrequency ablation may be used to slow tumor growth or destroy cancer cells; removing part of the liver or a liver transplant may be options if the tumors have spread.

Medications may be used to control excessive acid production. Often the first line of treatment for Zollinger-Ellison syndrome is to decrease acid production with drugs called proton pump inhibitors.

Other hormone-based medications may be used to counteract the acid-producing effects of gastrin.