Zollinger-Ellison syndrome is a tumorous disease in which tumors in the gastrointestinal tract cause overproduction of the hormone gastrin. In just over 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. An overproduction of this hormone manifests itself in a paraneoplastic appearance and is known as Zollinger-Ellison syndrome. This phenomenon is named after the US surgeons Zollinger and Ellison, who first described the complex of symptoms in the 20th century. For meanings of peroneal paralysis, please visit polyhobbies.com.
Pancreatic tumors are among the main symptoms of the syndrome. The upper small intestine is also often affected by tumors. In just over half of the cases, the tumors are malignant. About ten out of a million people are affected by the disease every year. This makes Zollinger-Ellison Syndrome an extremely rare disease that occurs predominantly between the ages of 30 and 60. Theoretically, younger people can also be affected by the symptoms of Zollinger-Ellison syndrome.
In Zollinger-Ellison syndrome, tumors develop in the pancreas and in the gastrointestinal tract. The growths produce gastrin, a hormone of 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 cases, gastrinomas are malignant and metastasize to the lymph nodes or liver.
About a quarter of all patients with Zollinger-Ellison syndrome are affected by multiple endocrine neoplasia type I and have multiple tumors. In addition to the pancreas, the pituitary and parathyroid glands are usually affected. Gastrin stimulates the production of gastric acid and causes the gastric parietal cells to release hydrogen ions. This increases the acid concentration in the stomach. As a result, gastric ulcers and duodenal ulcers occur.
Symptoms, Ailments & Signs
The Zollinger-Ellison syndrome causes severe abdominal pain, which preferably occurs in an atypical localization. Patients are often affected by reflux. In this phenomenon, stomach contents belch up into 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.
This results in high losses of electrolytes and vitamins. Some sufferers also vomit blood. The overproduction of gastric juice inactivates the patient’s lipases. Since these enzymes are indispensable for fat digestion, fats sometimes cannot be adsorbed by the small intestine and end up in the large intestine. The result is a greasy change in the stool consistency. Sometimes the pH value in the blood of those affected rises. 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 the Zollinger-Ellison syndrome. A concentration of more than 1,000 ng per liter is almost definitive for the diagnosis. If chromogranin can also be detected in the serum, this indicates neuroendocrine tumors. Therapy-resistant abdominal pain in an atypical location supports the diagnosis.
Imaging procedures such as octreotide scintigraphy, positron emission tomography, MRI, or CT are used to localize the tumors. Since the symptoms of the Zollinger-Ellison syndrome can also occur in the context of a MEN syndrome, this disease must be given special attention in the differential diagnosis.
In the case of Zollinger-Ellison syndrome, the prognosis depends on the degree of malignancy of the tumors and the presence of metastases. 90 percent of all patients with lymphatic metastases are still alive five years later, which corresponds to a favorable prognosis. Liver and pancreas metastases are considered prognostically unfavorable.
When should you go to the doctor?
Abdominal pain, swelling, ulcers, or changes in body shape need to be evaluated by a doctor. These are alarm signals from the organism, the cause of which should be determined. If these irregularities persist over a longer period of time or if the symptoms become more intense, a doctor must be consulted.
Disorders of the gastrointestinal tract, diarrhea and general abdominal pain must be evaluated and treated. Since the affected person is threatened with premature death without medical care in the event of a severe course of the disease, a doctor must be consulted at the first irregularities.
Digestive disorders, heartburn and inflammation of the larynx are further warning signals from 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 pressure pain, disturbances of sensitivity and inner restlessness, the symptoms should be clarified. If behavioral problems are noticed, if there is irritability or if there is a loss of appetite or weight loss, a doctor must be consulted. If bleeding occurs while going to the toilet, consult a doctor 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, the patients have to appear regularly for check-ups throughout their lives. Since it is not usually possible to surgically remove all tumor cells, radiation may also take place after the operation.
Since radiation has proven to be less effective for gastrinomas, this step is not recommended by all doctors. If the tumors are benign, they are still removed if possible, since 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, symptomatic therapy, which can at least prolong life, is carried out in addition to appropriate surgery. Survival is possible for decades. In order to improve the quality of life of the patients, their abdominal pain can also be treated by administering painkillers and thus alleviated if necessary. Diarrhea and the eruption of ulcers can also be prevented therapeutically.
The Zollinger-Ellison-Syndrome can be prevented at least in moderation by cancer screening.
In most cases, those affected with Zollinger-Ellison syndrome have only limited and few measures and options for direct aftercare available, so ideally the affected person should see a doctor at the first signs and symptoms of this disease. The sooner a doctor is consulted, the better the further course of this disease is usually.
If you want to have children, genetic testing and counseling is recommended to prevent the Zollinger-Ellison syndrome from recurring. A complete cure of the disease cannot be achieved because it is a genetic condition. When treating Zollinger-Ellison syndrome, most of those affected rely on physiotherapy and physiotherapy to relieve the symptoms.
Repeating the exercises at home can positively influence 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 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. A change in diet is important. It is important to avoid irritating foods and drinks in order to prevent the typical heartburn. Smokers should stop smoking. The consumption of coffee or alcohol must also be reduced. It is important to prevent heartburn and avoid subsequent 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 adhering to the medical instructions.
Strict bed rest and avoidance of stress apply after surgery. The diet should be continued. 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, you should continue to look out for any unusual symptoms. If stomach problems occur again, the doctor must be consulted. The gastroenterologist is responsible and can give patients with Zollinger-Ellison syndrome further self-help tips.