Zollinger-Ellison Syndrome: Causes, Symptoms, Treatment, Prognosis, Life Expectancy

About Zollinger-Ellison Syndrome

Zollinger-Ellison Syndrome is a complicated medical condition in which there is formation of multiple tumors in the pancreas or the duodenum. These tumors are called as Gastrinomas. These tumors secrete huge amounts of gastrin, which is a hormone responsible for producing acid excessively in the stomach which in turn causes peptic ulcers. This disease is pretty rare, but may occur at any point in life. Zollinger-Ellison Syndrome is usually diagnosed between the age range of 35-50. The standard treatment for Zollinger-Ellison Syndrome is treating the peptic ulcers and medications to reduce the amount of acid present in the stomach.

Zollinger-Ellison Syndrome

What Are The Causes Of Zollinger-Ellison Syndrome?

The root cause of Zollinger-Ellison Syndrome is not known, but how this syndrome develops is quite significant. This disease begins with the development of tumors in the pancreas or duodenum. There may also be tumors in the lymph nodes adjoining the pancreas. The pancreas is situated behind the stomach and is responsible for producing enzymes that help in digestion. The pancreas also produces hormones, which help in controlling acid formation in the stomach. The juices secreted by the pancreas, duodenum, and the liver get mixed in the duodenum. This is where maximum digestion of food takes place. The tumors formed in Zollinger-Ellison Syndrome are constituted of cells that produce excessive gastrin, as a result of which there is excessive gastric production in the stomach. The excessive production of acid then results in formation of peptic ulcers. These tumors may at times become malignant. The growth of the tumor may be slow but the spread of the cancer in the adjoining areas like the lymph nodes or the liver may be rapid.

What Are The Symptoms Of Zollinger-Ellison Syndrome?

Symptoms of Zollinger-Ellison Syndrome Include:

  • Pain in the abdomen
  • Persistent diarrhea
  • Burning and aching discomfort in the upper part of the abdomen
  • Presence of acid reflux
  • Heartburn
  • Nausea
  • Vomiting
  • Persistent weakness
  • Gastrointestinal bleeding
  • Unintentional weight loss
  • Poor appetite
  • Presence of anemia

How Is Zollinger-Ellison Syndrome Diagnosed?

To diagnose Zollinger-Ellison Syndrome, the treating physician will perform the following:

  • Medical History: The treating physician will take a detailed history and conduct a comprehensive physical examination. During history taking, the physician will ask about the signs and symptoms observed by the individual. In case there is a family history of Zollinger-Ellison Syndrome, then it may predispose that individual to this disease.
  • Blood Tests: The next step in diagnosis will be to take a blood sample and send it for analysis. The main aim of the analysis will be to look for elevated gastrin levels. In case there are elevated levels of gastrin, this might be suggestive of presence of tumors in the pancreas. The blood draw is usually done with the individual fasting. In case if the individual is on any medication to reduce acid formation then that medication might also need to be stopped before taking the blood sample. As there may be significant fluctuations in the level of gastrin, hence the test will have to be repeated a few times.
  • Gastrin Level Measurement: As elevated gastrin levels can be caused due to some other conditions also, the treating physician may also test the level of acid in the stomach to be clear as to what is causing elevated gastrin levels. Gastrin levels can also be elevated if an individual is on acid blocking medications or if the stomach is not making enough acid.
  • Secretin Stimulation Test: Another test that may be performed is the secretin stimulation test. In this test, the physician will first measure the gastrin levels and then inject a hormone called secretin and then test gastrin levels again. If the individual has Zollinger-Ellison Syndrome, then the levels of gastrin will significantly increase post injection of the hormone.
  • Upper Gastrointestinal Endoscopy: In this test, the physician will insert an instrument called endoscope which has a miniature camera inside it through the mouth to the stomach and look at the duodenum and stomach in detail observing for presence of ulcers. The physician may also take a tissue sample for biopsy to confirm the presence of gastrin producing tumors.
  • Imaging Studies: Imaging studies like nuclear scan, CT scan, MRI, and ultrasound may also be done to confirm the diagnosis of Zollinger-Ellison Syndrome
  • Endoscopic Ultrasound: In this procedure, the physician examines the stomach through an endoscope which is fitted with an ultrasound probe. This probe allows more detailed inspection of the whole digestive tract allowing easier detection of tumors if present.

What Are The Treatments For Zollinger-Ellison Syndrome?

For treatment of Zollinger-Ellison Syndrome, the physician will treat the tumors along with ulcers that are produced due to excessive acid production in the stomach. If the tumors are successfully removed then ulcer treatment becomes unnecessary. To remove the tumors in the pancreas, an experienced surgeon will need to be required as these tumors are extremely small and difficult to visualize. In case of a solitary tumor, this may be removed surgically but surgical removal is not recommenced for multiple tumors and those tumors that have spread to the adjacent areas. In order to control the growth of the tumor, the physician may recommend the following:

  • Removal of the tumor as much as possible
  • Attempts at killing the tumor by completely stopping the blood supply
  • Administrating medications directly into the tumor to cut down cancer risk
  • Utilizing chemotherapy to slow down the growth of the tumor
  • As a last resort, liver transplant may be done.

Treatments For Zollinger-Ellison Syndrome

What Is The Prognosis And Life Expectancy In Zollinger-Ellison Syndrome?

The tumors in cases of Zollinger-Ellison Syndrome become malignant in about 50% of cases. As stated, these tumors grow slowly but if malignant they rapidly spread to the adjoining areas like the lymph nodes and the liver. In case, if the tumor spreads to the liver then the prognosis becomes guarded and the life expectancy may be reduced to about eight years.

Pramod Kerkar, M.D., FFARCSI, DA
Pramod Kerkar, M.D., FFARCSI, DA
Written, Edited or Reviewed By: Pramod Kerkar, M.D., FFARCSI, DA Pain Assist Inc. This article does not provide medical advice. See disclaimer
Last Modified On:January 11, 2019

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