Zollinger-Ellison syndrome is a tumorous disorder in which tumors in the gastrointestinal tract cause overproduction of the hormone gastrin. In slightly 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 in a paraneoplastic appearance and is known as Zollinger-Ellison syndrome. The namesakes 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 among the leading symptoms of the syndrome. The upper small intestine is also frequently affected by tumors. In slightly more than half of the cases, the tumors are malignant. Approximately ten out of one million people are affected by the disease each year. This makes Zollinger-Ellison syndrome an extremely rare disease that occurs predominantly between the ages of 30 and 60. However, younger people can theoretically also be affected by the symptoms of Zollinger-Ellison syndrome.
Causes
In Zollinger-Ellison syndrome, tumors develop in the pancreas and gastrointestinal tract. The tumors produce gastrin, a hormone of the gastrointestinal tract. For this reason, the tumors are also called gastrinomas. These neuroendocrine tumors can appear singly or as multiple tumors. In slightly more than 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 as part of this. In addition to the pancreas, the pituitary and parathyroid glands are usually affected. Gastrin stimulates the production of gastric acid and causes the gastric vestibular cells to release hydrogen ions. In this way, the acid concentration in the stomach increases. As a result, gastric ulcers and duodenal ulcers occur.
Symptoms, complaints, and signs
Zollinger-Ellison syndrome causes severe abdominal pain that preferentially occurs in an atypical location. Patients are often affected by reflux. In this phenomenon, stomach contents bubble up in the esophagus. Stomach acid can inflame the mucous membranes of the anatomical structure and heartburn develops. 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 sufferers also vomit bloody vomit. The overproduction of gastric juice inactivates the patients’ lipases. Since these enzymes are irreplaceable for fat digestion, fats can sometimes no longer be adsorbed by the small intestine and pass into the colon. A fatty change in stool consistency is the result. Sometimes the pH value rises in the blood of the affected person. In rare cases, there is also an overproduction of parathyroid hormone. As a result, the calcium level in the blood is only regulated incorrectly.
Diagnosis and course of the disease
To diagnose Zollinger-Ellison syndrome, gastrin levels are determined. A concentration above 1 000 ng per liter is approximately diagnostic. If chromogranin can also be detected in the serum, this points to neuroendocrine tumors. Treatment-resistant abdominal pain in atypical localization supports the diagnosis. To localize the tumors, imaging techniques such as octreotide scintigraphy, positron emission tomography, MRI, or CT are ordered. Since the manifestations of Zollinger-Ellison syndrome may also occur in the setting of MEN syndrome, this condition requires special differential diagnostic consideration. The prognosis in Zollinger-Ellison syndrome depends on the malignancy grade 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 pancreatic metastases are considered prognostically unfavorable.
When should you see a doctor?
Abdominal pain, swelling, ulcers, or changes in body shape need to be evaluated by a physician.These are alarm signals of the organism, the cause of which should be determined. If these irregularities persist over a longer period of time or if the intensity of the complaints increases, a doctor must be consulted. Disorders of the gastrointestinal tract, diarrhea and general pain in the abdomen must be examined and treated. Since the affected person is threatened with premature death in case of a severe course of the disease without medical care, a doctor should be consulted already at the first irregularities. Disturbances in digestion, heartburn and inflammation of the larynx are further warning signals of the organism. If diffuse deficiency symptoms, a decrease in physical performance as well as dullness appear, the affected person needs medical help. In case of a pressure pain, disturbances of the sensibility as well as an inner restlessness, a clarification of the complaints should take place. If behavioral abnormalities are noticed, irritability is present or there is a loss of appetite as well as a loss of body weight, a doctor must be consulted. If bleeding occurs during toilet use, a physician should be consulted as soon as possible.
Treatment and therapy
Curative treatment is possible only in gastrinomas without metastases. In this case, the tumor or tumors are surgically removed as completely as possible, as far as this is within the realm of possibility. However, this does not rule out recurrences. Therefore, patients must attend regular check-ups throughout their lives. Since not all tumor cells can usually be removed surgically, the operation may be followed by additional radiation therapy. However, radiation has been shown to be less effective in gastrinomas, so this step is not recommended by all physicians. If the tumors are benign, they are still removed if possible because the risk of degeneration is high. Patients are also given octreotide to reduce gastrin secretion. Administration of proton pump inhibitors can usually decrease gastric acid production. If metastasis has already occurred, symptomatic therapy is given in addition to surgery as needed, which can at least prolong life. Survival is thus possible for decades. To improve the quality of life of patients, their abdominal pain can be additionally treated by the administration of painkillers and thus alleviated if necessary. Diarrhea and the rupture of ulcers can also be prevented therapeutically.
Prevention
Zollinger-Ellison syndrome can be prevented, at least in moderation, via cancer screening.
Follow-up care
In most cases, the person affected by Zollinger-Ellison syndrome has only limited and also only a few measures and options for direct aftercare at his or her disposal, so the person affected should ideally see a doctor at the first signs and symptoms of this disease. The sooner a doctor is consulted, the better the further course of the disease will usually be. If the patient wishes to have children, genetic testing and counseling is recommended to prevent a recurrence of Zollinger-Ellison syndrome. A complete cure of the disease cannot be achieved, as it is a genetic disease. In the treatment of Zollinger-Ellison syndrome, most sufferers rely on the measures of physical therapy and physiotherapy to relieve the symptoms. Repeating the exercises at home can positively influence the healing process. Furthermore, 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 affected person, although a general prediction cannot be made.
Here’s what you can do yourself
Treatment of Zollinger-Ellison syndrome can be helped by some self-help measures. A change in diet is important. Irritating foods and drinks should be avoided 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 secondary symptoms such as further irritation of the throat and stomach.The discomfort 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 doctor’s instructions. After surgery, strict bed rest and avoidance of stress apply. Diet should continue to be maintained. Once the tumor has been successfully removed, the symptoms of Zollinger-Ellison syndrome usually subside within a few days to weeks. Previously prescribed medications can be phased out in consultation with the physician. At the same time, the patient should continue to watch for unusual symptoms. If stomach complaints occur again, the doctor must be consulted. The gastroenterologist is responsible and can provide further self-help tips to patients with Zollinger-Ellison syndrome.
