Zollinger-Ellision Syndrome: Symptoms, Causes, Diagnosis, Treatment

Zollinger-Ellision Syndrome: Symptoms, Causes, Diagnosis, Treatment

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Zollinger-Ellision Syndrome: Symptoms, Causes, Diagnosis, Treatment

Zollinger-Ellison syndrome is a rare disease in which there are one or more tumors in upper part of small intestine or in pancrease (non beta cell tumor of pancreas), known as gastrinomas. These tumors secrete large amount of gastrin, which is responsible for production of gastric acid in stomach. Gastrin acts on Parietal cells of stomach glands (the Parietal cells) and cause increase production/secretion of hydrogen ions, which are used for production of gastric hydrochloric acid. In addition gastrin also cause hyperplasia of Parietal cells.

Due to production of excess stomach acid it leads to peptic ulcer, GERD, diarrhea and other symptoms of excess gastric acid. Zollinger-Ellison syndrome can occur any age in life, however more common at 20 years to 50 years of age. Treatment is usually symptomatic and aimed at healing ulcer and reducing stomach acid.

What are the signs and symptoms of Zollinger-Ellison syndrome?

The signs and symptoms of Zollinger-Ellison syndrome include:

  • Heartburn and acid reflux (GERD)
  • Pain in abdomen
  • Symptoms of excess gastric acid such as burning, aching, gnawing or discomfort in your upper abdomen
  • Diarrhea
  • Nausea and/or vomiting
  • Signs and symptoms of peptic ulcer
  • Bleeding in GIT (may cause melena)
  • Loss of appetite and weight loss

Are there any risk factors for Zollinger-Ellison syndrome?

If you have first degree blood relatives such as a sibling or parent, with MEN 1, you are at greater risk of developing Zollinger-Ellison syndrome.

What is the cause(s) of Zollinger-Ellison syndrome?

The exact cause of Zollinger-Ellison syndrome is unknown. However, how the problem occur is clear. It starts with appearance of a tumor (gastrinoma) in duodenum, pancreas or lymph node near pancreas. The tumors can be cancerous and spread to other organs of the body. Gastrinomas may be related with inherited condition called multiple endocrine neoplasia, type 1 (MEN 1). Approximately 25% of patients with gastrinoma have it as part of MEN-1.

Diagnosis of Zollinger-Ellison syndrome:

Diagnosis of Zollinger-Ellison syndrome involves following approach,

  • Thorough medical history of the symptoms and their appearance.
  • Blood test to check if there is high level of gastrin. However, high gastrin level is not always indicative of Zollinger-Ellison syndrome or gastrinoma, because this can also be caused by other medical conditions such as taking proton pump inhibitors (e.g. pantoprazole) for reducing acid production, or if stomach is producing less gastric acid for any reason. To make the better result the test can be done more than once.
  • Your doctor may also ask you to undergo another blood test a secretin stimulation test. For this test, gastrin level is checked first, and you are given an injection of the hormone secretin and gastrin level is measured again. If you have Zollinger-Ellison, your gastrin levels will increase even more.
  • Endoscopy of upper GIT, to look for ulcers in stomach and duodenum. Along with looking for ulcers, endoscopy may also be used for collecting samples for biopsy from suspected areas.
  • Endoscopic ultrasound is done by placing an ultrasound probe at the tip of endoscope. This procedure allows closer inspection and increase chance of spotting tumor easily.
  • Imaging: this include use of CT scan, MRI and other methods such as nuclear scanning somatostatin receptor scintigraphy.

Treatment of Zollinger-Ellison syndrome:

Treatment of Zollinger-Ellison syndrome involves treating the tumor and treating excess gastric acid.

  • Treating tumors (gastrinomas):

Treatment of tumor(s) involves surgical removal of tumor(s) by skilled surgeon, because tumors may be small and difficult to find exact location of tumor. If you have only single tumor surgical removal may be an easy option, however in case of multiple tumors in various locations (duodenum, pancreas etc.) may not be good option, especially if the tumor has spread to other organs such as liver. Your doctor may also decide to surgically remove the largest tumor from multiple tumors. Multiple tumors can be treated by cutting off blood supply to tumors, by using radiofrequency ablation, chemotherapy, injecting drugs in to the tumor.

  • Treatment of excess gastric acid:

The first line of treating excess gastric acid is use of proton pump inhibitors (such as pantoprazole, omeprazole etc.). They are highly effective in reducing gastric acid. Octreotide is a similar to hormone somatostatin, which may be useful in counteracting the effects of gastrin and be helpful, at least for some people suffering from Zollinger-Ellison syndrome.

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