Zollinger–Ellison Syndrome (ZES) is a rare disorder in which one or more tumours called gastrinomas form in the pancreas or upper small intestine (duodenum). These tumours release large amounts of a hormone called gastrin, which causes the stomach to produce excessive acid. The high levels of stomach acid can lead to peptic ulcers, severe heartburn, and damage to the stomach lining.
Causes and Prevalence
The condition may occur on its own or as part of a genetic disorder called Multiple Endocrine Neoplasia type 1 (MEN1), which causes tumours in multiple hormone-producing glands. Most gastrinomas are cancerous, though they grow slowly. The disease is more common in men and usually diagnosed between ages 30 and 60.
Symptoms
Patients with Zollinger–Ellison Syndrome often complain of severe and recurring ulcers that do not heal easily, even with medications. Symptoms may include burning abdominal pain, diarrhea, acid reflux, and unintentional weight loss. The ulcers may cause bleeding, vomiting, or blockage in the digestive tract if left untreated.
Diagnosis
Diagnosis starts with blood tests to measure the levels of gastrin, which are usually very high in this condition. To confirm the diagnosis, doctors may also perform imaging tests such as CT scans, MRI, or special endoscopic procedures to locate the tumours. Sometimes, a special scan called a somatostatin receptor scintigraphy (SRS) helps to find small tumours in the digestive tract or pancreas.
Treatment
The main goal of treatment is to reduce acid production and remove or control the gastrin-producing tumours. Medications called proton pump inhibitors (PPIs) are used to reduce stomach acid and help ulcers heal.
If the tumour is found and has not spread, surgery can be performed to remove it. In cases where the tumour has spread to other parts of the body, such as the liver or lymph nodes, treatment may include chemotherapy, targeted therapy, or long-term acid suppression.
Prognosis
Although ZES is a chronic condition, many patients live long lives with proper treatment. The outlook depends on whether the tumours are cancerous and if they have spread. Regular follow-ups and monitoring are necessary to check for tumour growth and manage symptoms over time.
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