Zollinger-Ellison Syndrome

The Zollinger-Ellison Syndrome is a tumorous disease in which tumors in the gastrointestinal tract cause an overproduction of the hormone gastrin. In a little more than half of all cases, the tumors are malignant gastrinomas. Curative therapy is possible before metastasis.

What is Zollinger-Ellison Syndrome?

The hormone gastrin is synthesized in the gastrointestinal tract. Overproduction of this hormone manifests itself in a paraneoplastic appearance and is called Zollinger-Ellison Syndrome designated. The namesake of this phenomenon are the US surgeons Zollinger and Ellison, who first described the complex of symptoms in the 20th century.

Tumors of the pancreas are one of the leading symptoms of the syndrome. The upper small intestine is also often affected by tumors. In a little more than half of the cases, the tumors are malignant tumors. The disease affects around ten out of a million people each year. This makes Zollinger-Ellison syndrome an extremely rare disease that occurs predominantly between the ages of 30 and 60 years. Theoretically, younger people can also be affected by the symptoms of Zollinger-Ellison syndrome.

causes

In Zollinger-Ellison syndrome, tumors develop in the pancreas and gastrointestinal tract. The growths produce gastrin, a hormone found in the gastrointestinal tract. For this reason, the tumors are also known as gastrinomas. These neuroendocrine tumors can appear individually or as multiple tumors. In just over half of the cases, gastrinomas are malignant and metastasize to the lymph nodes or the liver.

About a quarter of all patients with Zollinger-Ellison syndrome are affected by multiple endocrine neoplasia of type I and have multiple tumors as a result. In addition to the pancreas, the pituitary and parathyroid glands are usually affected. Gastrin stimulates the production of stomach acid and causes the parietal cells of the stomach to release hydrogen ions. In this way, the acid concentration in the stomach increases. As a result, gastric ulcers and ulcers of the duodenum occur.

Symptoms, ailments & signs

Zollinger-Ellison syndrome causes severe abdominal pain, which occurs primarily in an atypical location. The patients are often affected by reflux. In this phenomenon, stomach contents push up in the esophagus. Stomach acid can inflame the mucous membranes of the anatomical structure and cause heartburn. Laryngitis is also a common consequence of reflux. Diarrhea also occurs in half of the patients.

High losses of electrolytes and vitamins are the result. Some people also vomit bloodily. The overproduction of gastric juice inactivates the patient's lipases. Since these enzymes are indispensable for fat digestion, fats can sometimes no longer be adsorbed by the small intestine and end up in the large intestine. A greasy change in stool consistency is the result. Sometimes the pH rises in the blood of those affected. In rare cases, there is also an overproduction of the parathyroid hormone. As a result, the calcium level in the blood is only incorrectly regulated.

Diagnosis & course of disease

The gastrin level is determined for the diagnosis of Zollinger-Ellison syndrome. A concentration of more than 1,000 ng per liter is more or less confirming the diagnosis. If chromogranin can also be detected in the serum, then this indicates neuroendocrine tumors. Treatment-resistant abdominal pain in an atypical location supports the diagnosis.

In order to localize the tumors, imaging procedures such as octreotide scintigraphy, positron emission tomography, MRI, or CT are initiated. Since the symptoms of Zollinger-Ellison syndrome can also occur in the context of a MEN syndrome, this disease must be given special attention in the differential diagnosis.

The prognosis for Zollinger-Ellison syndrome depends on the degree of malignancy of the tumors and the presence of metastases. Ninety percent of all patients with lymph metastases are still alive five years later, which corresponds to a favorable prognosis. Liver and pancreatic metastases are considered to be poor prognostically.

When should you go to the doctor?

Abdominal pain, swelling, ulcers, or changes in body shape should be examined by a doctor. These are alarm signals from the organism, the cause of which should be determined. If these irregularities persist over a long period of time or if the symptoms become increasingly intense, a doctor must be consulted.

Gastrointestinal disorders, diarrhea, and general abdominal pain need to be investigated and treated. Since the person concerned is at risk of premature death without medical care in the event of a severe course of the disease, a doctor should be consulted as soon as the first irregularities occur.

Digestive disorders, heartburn and inflammation of the larynx are further warning signals of the organism. If there are diffuse deficiency symptoms, a decrease in physical performance and fatigue, the person concerned needs medical help. In the case of tenderness, sensitivity disorders and inner restlessness, the symptoms should be clarified. If behavioral problems are noticed, irritation is present or loss of appetite or loss of body weight occurs, a doctor must be consulted. If bleeding occurs while using the toilet, a doctor should be consulted as soon as possible.

Treatment & Therapy

Curative treatment is only possible for gastrinomas without metastases. The tumor or tumors are surgically removed as completely as possible, as far as this is possible. However, this does not rule out recurrences. Therefore, patients must appear regularly for check-ups throughout their life. Since not all tumor cells can usually be removed surgically, radiation may also take place after the operation.

However, since radiation has proven to be ineffective for gastrinomas, this step is not recommended by all doctors. If the tumors are benign, they are removed if possible because the risk of degeneration is high. Patients are also given octreotide to reduce gastrin secretion. The administration of proton pump inhibitors can usually reduce the production of gastric acid.

If metastasis has already taken place, an operation tailored to the needs takes place as well as symptomatic therapy that can at least extend the lifespan. Survival is possible for decades. In order to improve the patients' quality of life, their abdominal pain can also be treated with painkillers and, if necessary, alleviated. Diarrhea and the breakthrough of ulcers can also be prevented therapeutically.

prevention

The Zollinger-Ellison syndrome can be prevented at least to a limited extent through cancer prevention.

Aftercare

In most cases, those affected have only limited and only a few measures and options for direct follow-up care with Zollinger-Ellison syndrome, so that the person affected should ideally consult a doctor at the first signs and symptoms of this disease. The earlier a doctor is consulted, the better the further course of this disease will usually be.

If you want to have children, genetic testing and counseling is recommended in order to prevent Zollinger-Ellison syndrome from recurring. A complete cure of the disease cannot be achieved because it is a genetic disease. When treating Zollinger-Ellison syndrome, most people affected rely on physical therapy and physiotherapy to alleviate the symptoms.

Repeating the exercises at home can have a positive effect on the healing process. In addition, it is not uncommon for various medications to be taken. Those affected should pay attention to the prescribed dosage and regular intake. In most cases the syndrome by itself does not reduce the life expectancy of the person affected, and a general prediction cannot be made.

You can do that yourself

The treatment of Zollinger-Ellison syndrome can be supported by some self-help measures. It is important to change your diet. Irritant food and drinks should be avoided in order to prevent the typical heartburn. Smokers should quit smoking. The consumption of coffee or alcohol must also be reduced. It is important to prevent heartburn and secondary symptoms such as further irritation of the throat and stomach.

The symptoms can also be treated symptomatically, for example by placing a warm pillow on the painful areas. Gentle teas also have a soothing effect. The causal treatment of the tumor can be supported by the patient by following the medical guidelines.

After surgery, strict bed rest and avoidance of stress apply. The diet should continue to be maintained. If the tumor has been successfully removed, the symptoms of Zollinger-Ellison syndrome usually subside within a few days to weeks. Previously prescribed medication can be tapered off in consultation with the doctor. At the same time, attention should still be paid to any unusual symptoms. If stomach problems recur, a doctor should be consulted. The gastroenterologist is responsible, who can give patients with Zollinger-Ellison syndrome further self-help tips.