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Zollinger-Ellison Syndrome: Symptoms, Causes, and Treatment

28 September, 2020 Richa Tiwari

Zollinger-Ellison syndrome is an infrequent condition that is characterized by the formation of one or more tumors inside your pancreas or the upper side of your small intestine (duodenum). These tumors are known as gastrinomas because they secrete a large amount of gastrin hormone. And due to this, your stomach produces acid in bulk which then leads to peptic ulcers, diarrhea, and other medical conditions. 

Zollinger-Elison syndrome (ZES) can occur at any stage in life but most of the people who get affected by it are between the ages of 20 – 50. The general treatment done for ZES consists of treating stomach acid and peptic ulcers. 

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Symptoms of Zollinger-Ellison Syndrome

The commonly experienced signs and symptoms in the Zollinger-Ellison Syndrome are: 

  • Pain in the abdomen
  • Diarrhea
  • Burning, aching, and discomfort in the upper side of your abdomen
  • Heartburn
  • Acid Reflux
  • Nausea or Vomiting
  • Bleeding inside your digestive tract
  • Weight loss for no reason
  • Loss of appetite

You should visit the doctor and seek medical consultation as soon as possible if there is a persistent burning or gnawing pain in your abdomen, and along with these you’re also experiencing diarrhea, vomiting, and hyperacidity. 

Causes of ZES

The exact cause behind the occurrence of Zollinger-Ellison Syndrome is yet to be discovered. But how the events happen in this condition is known. It all starts with a tumor or tumors that form in the pancreas, duodenum, or the lymph nodes that are adjacent to the pancreas. The work of the pancreas is to produce enzymes that are necessary for digesting food. It also produces hormones like insulin which helps in controlling your blood glucose. 

These digestive juices produced by the pancreas, liver, and gallbladder get mixed in the duodenum, it is the point where digestion reaches its highest. 

The tumors that occur in this syndrome are made of cells that secrete a huge amount of gastrin hormone. This increased gastrin forces the stomach to produce an extremely large amount of acid and this excessive acid leads to peptic ulcers and even diarrhea.  

These tumors are also cancerous in nature, although the speed of growth of tumors is slow, they can spread anywhere else like most commonly to nearby lymph nodes or your liver. 

Risk Factors of ZES

If you have someone in your blood relation like a sibling or your parent with a condition known as MEN1, then your chances of developing Zollinger-Ellison Syndrome increases. MEN1 is an inherited condition, its full form is Multiple Endocrine Neoplasia, type 1, in which people have tumors in parathyroid and pituitary glands also. 

How Zollinger-Ellison Syndrome is Diagnosed? 

Your doctor will look for your signs and symptoms and your medical history. A blood test will be taken to analyze the sample of your blood and gastrin levels. An elevated amount of gastrin indicates tumors in your pancreas or duodenum. Other tests which are done are:

  • Upper gastrointestinal endoscopy
  • Endoscopic Ultrasound
  • Imaging tests – It helps in locating tumors.

Treatment of Zollinger-Ellison Syndrome

Treatment of ZES consists of curing the hormone-secreting tumors along with the treatment of ulcers they cause. 

Treatment of tumors

The surgery done for removing the tumors requires an expert surgeon because the tumors are mostly small and difficult to locate. If there is only one tumor then it can be removed surgically but if you have multiple tumors spread across your liver then surgery may not be an option. But still, doctors recommend removing one large tumor. Some other treatments that are advised by doctors are: 

  1. Removing tumor that is inside of the liver
  2. Destroying the tumor by stopping or cutting off the blood supply(embolization) or using heat to destroy cancer cells
  3. Injecting drugs inside the tumor to decrease the cancer symptoms
  4. Chemotherapy for slowing the tumor growth
  5. A liver transplant 

Treatment of excessive acid

To stop excess acid production medications known as proton pump inhibitors are used first. Some commonly prescribed medicines are lansoprazole(Prevacid), omeprazole(Prilosec Zegerid), rabeprazole(Aciphex), pantoprazole(Protonix), and esomeprazole(Nexium).  

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Zollinger-Ellison Syndrome is a rare disease which can occur at any time in life, especially between the age of 20-50 years. In this tumor or tumors that secrete gastrin hormone forms inside your pancreas or duodenum. The treatment usually consists of removing the tumors and treating the excessive acid produced due to this condition.