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Zollinger–Ellison syndrome (ZES)

This topic covers various aspects of Zollinger–Ellison syndrome.

Zollinger-Ellison syndrome (ZES) is a disease of the gastrointestinal system. People who have ZES develop tumors known as gastrinomas in the pancreas and duodenum (the first section of the small intestine). The gastrinomas caused by ZES secrete the hormone gastrin. Because gastrin creates excessive stomach acid, patients with ZES can also develop stomach and duodenal ulcers.

Causes

The exact cause of Zollinger-Ellison syndrome remains unknown. But the sequence of events that occurs in Zollinger-Ellison syndrome is clear. The syndrome begins when a tumor (gastrinoma) or tumors form in your pancreas, duodenum or the lymph nodes adjacent to your pancreas.

The tumors that occur with Zollinger-Ellison syndrome are made up of cells that secrete large amounts of gastrin, which in turn causes the stomach to produce far too much acid. The excessive acid then leads to peptic ulcers and sometimes to diarrhea.

Besides causing excess acid production, the tumors may be cancerous (malignant). The tumors themselves grow slowly, but the cancer can spread elsewhere - most commonly to nearby lymph nodes or your liver.

Zollinger-Ellison syndrome may be caused by an inherited condition called multiple endocrine neoplasia, type I (MEN I). People with MEN I have multiple tumors in the endocrine system in addition to pancreatic tumors.

Symptoms

People who have Zollinger-Ellison syndrome don't always have symptoms.

When symptoms do occur, they include:

  • Pain in the esophagus, especially between and after meals at night Nausea
  • Diarrhea
  • Vomiting
  • Bleeding from the stomach
  • Weakness
  • Fatigue
  • Wheezing

Tests and diagnosis

  • Secretin stimulation test
  • Blood tests
  • Gastrin level measurement
  • Upper gastrointestinal endoscopy
  • Nuclear scan, CT Scan, ultrasound or MRI
  • CAT Scan
  • Endoscopic ultrasound

Treatments and drugs

ZES is treated by reducing the amount of acid your stomach produces. Medications called proton pump inhibitors are usually prescribed. These drugs, which include lansoprazole (Prevacid), omeprazole (Prilosec), pantoprazole (Protonix), esomeprazole (Nexium), and rabeprazole (Aciphex), curb the production of stomach acid and allow the ulcers to heal.

Doctors may also prescribe medications known as H2-blockers, such as cimetidine (Tagamet), famotidine (Pepcid), and ranitidine (Zantac). However, these drugs don't work as well to reduce stomach acid.

In some cases, a patient may need surgery for the peptic ulcers or to remove the gastrinoma. However, only about 30%-40% of patients who have surgery are cured. For malignant tumors, radiation and chemotherapy may be offered.

Outlook

Gastrinomas tend to grow slowly and are not always malignant. The five-year survival rate depends on whether tumors are cancerous and if they've spread. If they have not spread to the liver, the fifteen-year survival rate is approximately 83%. This drops to a ten year survival of about 30% if the tumors have spread to the liver.

Source : Portal Content Team

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