Zollinger-Ellison Syndrome, a rare type of disease, is seen in only one in every 1 million people. Although the disease can be seen in both male and female sexes and in all age groups, it has been determined that the rate of catching this disease is higher in male individuals between the ages of 30-50. This disease, which is thought to be caused by genetic factors, requires early diagnosis and treatment as it can cause great damage to the digestive tract.
What causes Zollinger-Ellison syndrome?
Experts aren't entirely sure what causes ZES. In most cases, the disorder occurs for no apparent reason. In 25 percent of cases, it is associated with an inherited, tumor-causing condition called multiple endocrine neoplasia type 1 (MEN1). More research is needed to better understand this situation. It is more common in men than women and typically occurs between the ages of 30 and 50.
What are the symptoms of Zollinger-Ellison syndrome?
The symptoms of Zollinger-Ellison syndrome are similar to those of excess stomach acid, peptic ulcer disease, and reflux. Therefore, the symptoms of the disease can be confused with these ailments. Apart from all these symptoms, the findings of Zolinger-Ellison syndrome are as follows:
- Diarrhea, which may be the only symptom
- Burning pain in the abdomen or abdomen between the navel and the middle of the chest
- Painful burning sensation in the chest
- Nausea or vomiting
- Weight loss
- Chest pain
- Resistant vomiting
- Breathing problems
- Pain while swallowing
- Symptoms of bleeding in the digestive tract
- Sudden, sharp stomach pain that doesn't go away
- Weakness or feeling faint
How is Zollinger-Ellison syndrome diagnosed?
If your doctor suspects ZES, they will order a blood test to check for high gastrin levels. If excess gastrin is detected, imaging tests will be done to determine the size and location of the gastrinomas. These tests may include:
- CT scan
- Magnetic resonance imaging
- Endoscopic ultrasound
If you need an endoscopic ultrasound, your throat may be numbed with a liquid anesthetic, or you may receive a mild sedative at an outpatient center.
How is Zollinger-Ellison syndrome treated?
Your doctor may recommend a variety of treatments.
Proton Pump Inhibitors: Your doctor may recommend drugs called proton pump inhibitors. These can be used to help reduce the amount of stomach acid produced. This, in turn, can help ulcers heal. These drugs are usually given in much higher doses to people with ZES than to people with ulcers without gastrinoma. You will need careful monitoring while taking these medications. Long-term use of proton pump inhibitors can increase your risk of hip, wrist, and spine fractures, especially after age 50.
Surgery: In some cases, surgery may be needed to remove tumors. Surgery can be difficult because many gastrinomas are small, difficult to locate, and present in multiple conditions. If your doctor decides that you are a candidate for this type of treatment, they will discuss the details of your surgery.
Chemotherapy: Your doctor may also recommend chemotherapy to shrink tumors, or treatments like these to kill cells and stop tumors from growing.
How long does a Zollinger Ellison Syndrome patient live?
The prognosis is very good in patients with Zollinger-Ellison syndrome without metastatic disease. Early diagnosis usually results in more than 80% survival at 15 years. In general, patient effects and mortality rates of ZES are low due to good medical and surgical management. Less than 5% of patients develop a complication such as perforation of the organs, stenosis of the gastric outlet, or esophageal stricture.
Is Zollinger-Ellison syndrome a genetic disease?
As mentioned above, in patients with Zollinger-Ellison syndrome, a rare genetic disorder called MEN1 causes gastrinomas in approximately 20–25% of cases. MEN1 is a disease involving varying combinations of more than 20 endocrine and non-endocrine tumors. Booms can be parathyroid tumors, pituitary tumors, or gastrointestinal tumors. Zollinger Ellison Syndrome can also be included in this spectrum. A MEN1 gene analysis should be performed to confirm the diagnosis of MEN1.