Zollinger-Ellison Syndrome: Causes, Symptoms, Risk Factors, Diagnosis and Treatment

Zollinger-Ellison syndrome is an uncommon condition characterised by one or more tumours arising in the upper portion of the small intestine or pancreas. Gastrinomas are the term for the tumours. The hormone gastrin is produced in huge quantities by these gastrinomas. 

Gastrin produces excessive amounts of stomach acid, which can result in peptic ulcers. Abdominal pain and diarrhoea are two more symptoms that may result from high gastrin levels. Anyone can develop Zollinger-Ellison syndrome at any point in their lives. However, between the ages of 20 and 60 is typically when people become aware of their ailment. 

An integral aspect of the standard treatment plan is medication to treat ulcers and reduce stomach acid. Tumours may occasionally also need to be surgically removed.

Causes Of Of Zollinger-Ellison Syndrome

It is unknown what specifically causes Zollinger-Ellison syndrome. However, the sequence of events in Zollinger-Ellison syndrome usually follows the same pattern. Tumours in the pancreas or the duodenum, a portion of the small intestine, are the first sign of the syndrome. 

The part that connects to your stomach is called the duodenum. Tumours can also develop in other locations, like the lymph nodes near your pancreas. Located beneath and behind your stomach is your pancreas. It produces the enzymes required for food digestion. Insulin is among the several hormones produced by the pancreas. Insulin, commonly known as glucose, is the hormone that aids in blood sugar regulation. 

Pancreatic, gallbladder, and liver digesting fluids mix in the duodenum of the stomach. This is where most of your digestion happens. Tumours associated with Zollinger-Ellison syndrome are composed of cells that secrete a large amount of the hormone gastrin. 

They are occasionally referred to as gastrinomas as a result. Increased gastrin causes the stomach to create much too much acid. Peptic ulcers and occasionally diarrhoea are the result of too much acid in the stomach.

Not only do the tumours produce excessive amounts of acid, but they are often cancerous. Even though tumours typically grow slowly, cancer can nonetheless spread to other parts of the body, most commonly the liver or nearby lymph nodes.

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Correlation to MEN 1

The inherited condition known as multiple endocrine neoplasia, type 1 (MEN 1) could be the source of Zollinger-Ellison syndrome. In those with MEN 1, parathyroid gland tumours are also prevalent. They might also have tumours in their pituitary glands.

MEN 1 gastrinomas are present in about 25% of patients with gastrinomas. Additionally, they may have malignancies in other organs, like the pancreas.

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Symptoms Of Zollinger-Ellison Syndrome

Symptoms of Zollinger-Ellison syndrome can include the following:

• Abdominal pain
• Diarrhea
• Burning or painful sensation in the upper abdomen
• Acid reflux and heartburn
• Nausea 
• Burping 
• Vomiting
• Internal bleeding in the digestive tract
• Losing weight effortlessly
• Decrease in appetite

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Risk Factors Of Zollinger-Ellison Syndrome

If you have a parent or sibling who is a first-degree relative who has MEN 1, you are more likely to get Zollinger-Ellison syndrome.

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Diagnosis Of Zollinger-Ellison Syndrome

Your doctor's diagnosis usually depends on one or more of the following:

• Medical history: Inquiring about your symptoms, your doctor goes over your medical history.
• Blood testing: A blood sample is obtained to determine whether you have increased levels of gastrin. Raised gastrin levels can be a sign of pancreatic or duodenal tumours, but they can also be caused by other illnesses. For example, your level of gastrin may be higher if you have had gastric surgery or if you do not have acid in your stomach. Furthermore, gastrin levels can rise when taking drugs that reduce acid. You are required to fast before this test. It may also be necessary for you to stop taking drugs that decrease acid. Due to the frequent fluctuations in gastrin levels, this test may be repeated multiple times.
• Secretin stimulation test: The hormone that regulates stomach acid is called secretin. Before doing this test, your doctor takes your gastrin levels. You'll then be given an injection of secretin. We'll take another look at your gastrin levels. The amount of gastrin will rise sharply when Zollinger-Ellison is present.
• Upper gastrointestinal endoscopy: For this exam, you must be sedated. A thin, flexible instrument is sent down the throat and into the stomach and duodenum during an endoscopy. This instrument is known as an endoscope. There's a light and a camera at the end. It helps the physician to look for ulcers. During the endoscopy, tissue samples might be taken out. This is known as a biopsy. And whether the stomach is producing acid can also be found via an endoscopy. If the patient has elevated gastrin levels and acid reflux, a diagnosis of Zollinger-Ellison may be made. You will be expected to fast after midnight the night before the exam.
• Ultrasound-guided endoscopy: This procedure makes use of an endoscope that has an ultrasonic probe attached. Gastric, duodenal, and pancreatic tumours can all be found more easily when the probe is used.
• Biopsy: A tissue sample could be taken by your healthcare provider using an endoscope. For this test, a midnight fast is required in addition to sedation.
• Imaging tests: Your healthcare provider may utilise imaging techniques to look for tumours. Tests using nuclear scans include somatostatin receptor scintigraphy. This test uses radioactive tracers to find tumours. Other helpful imaging modalities include computerised tomography (CT), magnetic resonance imaging (MRI), Ga-DOTATATE PET-CT scanning, and ultrasound.

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Treatment Of Zollinger-Ellison Syndrome

Treatment of the hormone-secreting tumours and the ulcers they induce are the two main objectives in the management of Zollinger-Ellison syndrome.

Treatment for tumours

The procedure to remove the gastrinomas requires a skilled surgeon because the tumours are often small and difficult to find. If you only have one tumour, your doctor may be able to remove it surgically. 

Surgery might not be an option for you, though, if you have several tumours or cancers that have spread to your liver. However, if you have multiple large tumours, your surgeon may still recommend removing one of them.

In some cases, doctors recommend further treatments to halt the growth of the tumour, like:

• Debulking is the technique of removing as much of a liver tumour as possible.
• Embolisation is an effort to cut off the tumour's blood supply to kill it.
• Using heat to destroy cancer cells via a method called radiofrequency ablation.
• Pharmaceutical injections into the tumour to address the symptoms of malignancy.
• Using chemotherapy to try and stop the tumour's growth.
• Having a liver transplantation.
• Excessive acid handling

The overproduction of acid can almost always be controlled. The first line of treatment is a proton pump inhibitor drug. When taken by patients with Zollinger-Ellison syndrome, these drugs effectively lower the production of acid.

Proton pump inhibitors are potent drugs which decrease acid. They work by preventing the tiny "pumps" found inside acid-secreting cells from doing their job. Recommendations for drugs include lansoprazole, omeprazole , pantoprazole , rabeprazole , and esomeprazole.

There is an increased chance of hip, wrist, and spine fractures when using prescription proton pump inhibitors for an extended period. Individuals over 50 are especially at risk. But given the benefits of acid-blocking, this small risk should be taken into account.

To reverse the effects of gastrin, some people may find relief from the medicine octreotide, which functions similarly to the hormone somatostatin.

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