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Zollinger-Ellison syndrome is an uncommon disorder in which one or more tumors develop in your pancreas or upper small intestine (duodenum). These tumors, known as gastrinomas, secrete a huge amount of the hormone gastrin, which causes your stomach to create an excessive amount of acid. The excess acid subsequently causes peptic ulcers, diarrhea, and other symptoms.
Zollinger-Ellison syndrome can strike at any age, however most patients are diagnosed between the ages of 20 and 60. Zollinger-Ellison syndrome is typically treated with medications that lower stomach acid and cure ulcers.
WHAT ARE THE SYMPTOMS OF ZOLLINGER-ELLISION SYNDROME?
Among the signs and symptoms of Zollinger-Ellison syndrome are:
- Pain in the abdomen
- Diarrhea
- Vomiting and nausea
- Bleeding in your digestive tract
- Unintentional weight loss
- Upper abdominal burning, hurting, gnawing, or discomfort
- Heartburn and acid reflux
- Burping
- Reduced appetite
WHAT ARE THE CAUSES OF ZOLLINGER-ELLISON SYNDROME?
The cause of Zollinger-Ellison syndrome is yet unknown. However, the sequence of events in Zollinger-Ellison syndrome is well-defined. When one or more tumors (gastrinomas) grow in your pancreas or duodenum, or at other places such as lymph nodes near your pancreas, the condition begins.
Your pancreas is located behind and beneath your stomach. It creates enzymes that are required for food digestion. The pancreas also generates various hormones, including insulin, which aids in blood glucose regulation.
Digestive juices from the pancreas, liver, and gallbladder mingle in the duodenum, which is located next to the stomach in the small intestine. This is when digestion is at its most efficient.
Zollinger-Ellison syndrome tumors are formed up of cells that secrete a significant amount of the hormone gastrin. When gastrin levels rise, the stomach produces far too much acid. Excess acid causes peptic ulcers and, in certain cases, diarrhea.
Aside from creating excessive acid production, tumors are frequently carcinogenic (malignant). Although the tumors develop slowly, the cancer can spread to other parts of your body, most commonly to adjacent lymph nodes or your liver.
Zollinger-Ellison syndrome association with MEN 1
Multiple endocrine neoplasia, type 1 is thought to be the etiology of Zollinger-Ellison syndrome (MEN 1). Tumors in the parathyroid glands and pituitary glands are also common in people with MEN 1.
Men 1 is present in around 25% of persons with gastrinomas. Tumors in the pancreas and other organs are also possible.
WHAT ARE THE RISK FACTORS FOR ZOLLINGER-ELLISON SYNDROME?
Males aged 25–50, as well as persons who have a parent with the illness, are at a higher risk for Zollinger-Ellison syndrome. Zollinger-Ellison syndrome is incurable.
WHAT ARE THE COMPLICATIONS OF ZOLLINGER-ELLISON SYNDROME?
The severity of the peptic ulcers and the overproduction of stomach acid are linked to Zollinger-Ellison syndrome complications. Peptic ulcers that are severe can:
- Penetrate an artery, resulting in internal bleeding.
- Cause swelling, which causes a blockage in the passage of food leaving the stomach.
- Induce intestinal perforation (hole in the wall of the stomach and or duodenum).
The following Zollinger-Ellison syndrome consequences can be fatal. If you have any of the following symptoms, get emergency medical attention:
Stools that are black or bloody
Chest ache
Symptomatic anemia (tiredness, lightheadedness, dizziness, hypotension, rapid heart rate, etc.).
Stomach pain that is severe, abrupt, and/or continuous.
Vomit that resembles coffee grounds or contains blood
HOW IS ZOLLINGER-ELLISON SYNDROME DIAGNOSED?
Your doctor will base his or her diagnosis on the following factors:
- Medical history: Your doctor will inquire about your signs and symptoms, as well as go over your medical history.
- Blood tests: A sample of your blood is tested to check if you have high gastrin levels. While excessive gastrin levels may indicate pancreas or duodenal tumors, they can also be caused by other illnesses. Gastrin levels may be raised if your stomach isn't producing acid or if you're on acid-reducing drugs, such as proton pump inhibitors.
To acquire the best accurate measurement of your gastrin levels, you should fast before this test and avoid taking any acid-reducing drugs. Because gastrin levels change, this test may need to be done several times.
A secretin stimulation test may also be performed by your doctor. Your doctor will first measure your gastrin levels, then inject you with the hormone secretin and measure your gastrin levels again. If you have Zollinger-Ellison syndrome, your gastrin levels will rise even higher.
- Endoscopic ultrasound: During this operation, your doctor uses an endoscope equipped with an ultrasound probe to check your stomach, duodenum, and pancreas. The probe allows for a more thorough examination, making it easier to detect malignancies.
A tissue sample can also be extracted using the endoscope. You must fast after midnight the night before the exam, and you will be sedated during it.
- Imaging tests: Your doctor may utilize imaging techniques such as somatostatin receptor scintigraphy, a nuclear scan. This test employs radioactive tracers to aid in the detection of cancers. Ultrasound, computerized tomography (CT), magnetic resonance imaging (MRI), and Ga-DOTATATE PET-CT scanning are all useful imaging techniques.
- Upper gastrointestinal endoscopy: After you've been sedated, your doctor will introduce an endoscope, which is a small, flexible equipment with a light and video camera, down your throat and into your stomach and duodenum to look for ulcers. Your doctor may remove a tissue sample (biopsy) from your duodenum using an endoscope to help determine the presence of gastrin-producing tumors. You will be instructed by your doctor not to eat anything after midnight the night before the test.
WHAT ARE THE TREATMENTS FOR ZOLLINGER-ELLISON SYNDROME?
Treatment for Zollinger-Ellison syndrome focuses on both the hormone-secreting tumors and the ulcers they induce.
Tumor treatment
Because the tumors in Zollinger-Ellison are often small and difficult to identify, removing them takes the expertise of a trained surgeon. If you only have one tumor, your doctor may be able to remove it surgically; however, if you have numerous tumors or tumors that have progressed to your liver, surgery may not be an option. However, even if you have many tumors, your doctor may still advise you to have a single huge tumor removed.
In some circumstances, doctors recommend additional treatments to inhibit tumor growth, such as:
- Getting rid of as much of a liver tumor as possible (debulking)
- Attempting to kill the tumor by cutting off its blood supply (embolization) or by employing heat to kill cancer cells (radiofrequency ablation)
- Injecting medicines into the tumor to alleviate cancer symptoms
- Using chemotherapy to try to decrease tumor growth
- Getting a liver transplant
Excess acid treatment
Excess acid production is almost always manageable. The first line of treatment is with medications known as proton pump inhibitors. These drugs are useful in reducing acid production in Zollinger-Ellison syndrome.
Proton pump inhibitors are potent medications that lower acid levels by inhibiting the operation of small "pumps" within acid-secreting cells. Lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), rabeprazole (Aciphex), and esomeprazole are all often prescribed drugs (Nexium)
According to the Food and Drug Administration, long-term use of prescription proton pump inhibitors, particularly in those aged 50 and older, has been linked to an increased risk of hip, wrist, and spine fractures. This is a minor danger that should be balanced against the acid-blocking benefits of these drugs.
Octreotide (Sandostatin), a medicine comparable to the hormone somatostatin, may aid some people by counteracting the effects of gastrin.