Gastrinoma tumors found in the small intestine are known as the Zollinger-Ellison syndrome. Learn everything you need to know about this syndrome on time.
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 it 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 tumor is called gastrinoma.
The gastrinoma secretes 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 the causes of a gastrinoma?
Science does not know the precise reason why Zollinger-Ellison syndrome occurs. What we know 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.
Symptoms of Zollinger-Ellison syndrome
Zollinger-Ellison syndrome can cause symptoms such as the following:
- Pain in the abdomen: discomfort, burning sensation or excruciating pain
- Diarrhea
- Heartburn and reflux
- Reduced appetite
- Nausea and vomiting
- Involuntary weight loss
- Bleeding from the digestive tract
The symptoms of this condition are very similar to those of a peptic ulcer, but they are more persistent, but just as intense. Many times it is treated as if it were an ulcer disease, with drugs designed for it.
The problem is that some medications may mask symptoms and make the diagnosis more confusing. Therefore, the doctor should be informed if omeprazole, cimetidine or famotidine has been ingested for long periods.
Diagnosis and treatment
The diagnosis of Zollinger-Ellison syndrome depends on clinical symptoms, medical history and through some tests such as the following:
- Blood test: the goal is to evaluate gastrin levels. A high level is not conclusive, since there can be elevation in other pathologies.
- Gastrointestinal endoscopy: doctors insert an instrument consisting of a light and camera to explore the digestive tract, looking for ulcers. It also allows them to take a tissue sample or biopsy to detect tumors that produce gastrin.
- Endoscopic ultrasound: the endoscope carries a probe and makes it easier to detect tumors. It also allows doctors to take a tissue sample.
- Others: Doctors may order other tests, such as somatostatin receptor scintigraphy, ultrasound, CT scan, or MRI.
Therapeutic approach
Treatments of Zollinger-Ellison syndrome aim at both tumors and ulcers. It is not common to go to surgery to remove neoplasms, since they are usually very small and are located in different parts.
Typically, doctors treat the tumors with procedures such as embolization or radiofrequency ablation. Chemotherapy can also slow growth while drugs can relieve symptoms.
The first-line treatment to address excessive acid production is with drugs, proton pump inhibitors. These block the secretion of acid in the cells. Sometimes doctors prescribe a drug similar to the hormone somatostatin to counteract the effects of gastrin.
Possible complications of Zollinger-Ellison syndrome
The most serious complication is that the tumor spreads to other organs. It usually travels from the pancreas or duodenum to nearby lymph nodes or the liver.
Another serious complication is perforation or bleeding due to ulcers in the stomach and duodenum. Also, severe diarrhea and severe weight loss may occur.
Prognosis
The cure rate is low, even when doctors find the tumor early and can be remove it. Despite this, they grow slowly, so people live many years after getting the diagnosis.
The prognosis is less promising in the case of malignant tumors. If there is metastasis to the liver, only between 20 and 40% of those affected survive more than 10 years. People with MEN 1 have a high survival rate and only in 15% of cases does the disease have an aggressive course.
Zollinger-Ellison syndrome is the sign of something wrong
The prognosis for Zollinger-Ellison syndrome has improved significantly in the last 25 years. In the past, the leading cause of death was gastrointestinal bleeding and perforation, caused by ulcers.
Today drug treatment has significantly reduced mortality from these causes. Unlike other diseases, the most serious complication is metastasis to the liver and not to the lymph nodes. Therefore, its detection implies a risk that should not be overlooked.
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