Science still knows little about Zollinger-Ellison syndrome. Despite this, today this disease has a much better prognosis than in the past, thanks to the development of new treatments.
Zollinger-Ellison syndrome is a rare disease. It is estimated that it affects between 1 and 2 people per million inhabitants, being more common in women. It is usually diagnosed between the ages of 20 and 50, but can appear at any time in life.
The main characteristic is that there is excessive secretion of acid in the stomach. This leads to ulcers forming there. Zollinger-Ellison syndrome is considered a serious disorder and is often resistant to treatment.
What is Zollinger-Ellison syndrome?
Zollinger-Ellison syndrome is a condition in which one or more tumors form in the pancreas or duodenum, which is the upper part of the small intestine. These tumors are called gastrinomas .
Gastrinomas secrete a hormone called gastrin in large amounts. This leads to the stomach producing more acid than normal. The direct effect is the continuous production of peptic ulcers.
The tumors are small and slowly growing. In about 50% of cases these formations are malignant. Likewise, half of the patients with Zollinger-Ellison syndrome have multiple tumors.
Between 10 and 20% of those affected develop tumors in other parts of the body: stomach, splenic hilum, mesentery, lymph nodes or ovaries. About 40 to 60% of those with this condition also have a condition called multiple endocrine neoplasia .
What are your causes?
Science does not know the precise reason why Zollinger-Ellison syndrome occurs. What is known is that it begins with one or more tumors that secrete large amounts of gastrin. If they are malignant they can spread throughout the rest of the body.
Scientists have found a link between Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1 (MEN 1). This is a hereditary disease in which there is hyperactivity in the endocrine glands. This causes the characteristic tumors to form.
MEN 1 results from a defective gene that is associated with a protein called menin . A person who has blood relatives with this disease is more likely to develop Zollinger-Ellison syndrome.