Zollinger-Ellison syndrome is a symptom complex characterized by the appearance of peptic ulcers of the stomach and duodenum, accompanied by regular diarrhea. This disease was named after doctors who in 1955 discovered the relationship between pancreatic secretions, high acid production of gastric juice and the formation of peptic ulcers. In contrast to the classical clinical picture of Zollinger-Ellison syndrome, antiulcer therapy is ineffective. Next, we will look at what causes the disease and what treatment it requires.
What is Zollinger Ellison syndrome
Zollinger syndrome was first identified in 1955, when two patients were found to develop peptic ulcers in the upper digestive tract. This condition is caused by a developing hormonally active tumor in the pancreas - gastrinoma. The nature of gastrinoma is very aggressive; it produces excessive amounts of gastrin, which results in increased production of stomach acid and the development of stomach ulcers.
Gastrinomas belong to the group of adenomas with endocrine-like cells. A significant portion (seventy-five percent) of gastrinomas are malignant in nature and are characterized by slow growth with metastasis to:
- liver;
- spleen;
- The lymph nodes;
- mediastinum;
- peritoneum;
- skin covering.
Externally, the neoplasms are single or multiple dark red nodes with a dense consistency and rounded shape; more often they are not large in size and reach only 0.2-2 cm. The localization of formations most often during this syndrome is detected in the tail and body of the pancreas. In a third of patients with gastrinoma, the location is peripancreatic lymph nodes or the small intestine. Sometimes it is possible to form tumors in the spleen, liver tissues and stomach. The development of such a pathology is rare, only four cases per one million population. The disease most often affects men from twenty to fifty years of age.
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Etiology
The pathology received a similar name according to the names of the scientists who first described its clinical course. It is noteworthy that today the reasons for the development of a tumor that secretes large amounts of gastrin, which is a biologically active substance that has a regulating effect on the secretion of gastric juice, remain unknown.
However, against the backdrop of numerous scientific research conducted in the field of gastroenterology, a number of predisposing factors have become known that significantly increase the likelihood of developing such a disease. These include:
- burdened heredity;
- dysfunction of the endocrine system, in particular the adrenal glands and pituitary gland;
- hyperplasia of the parathyroid glands;
- the presence of ulcerative tumors in the small intestine or duodenum;
- constant entry into the intestines of large amounts of acidic gastric contents;
- benign or malignant tumors localized in the pituitary gland;
- impaired motility or inflammatory process in the small intestine;
- copious secretion of pancreatic bicarbonates.
In addition, there is an assumption that the etiology of this disease is multiple endocrine neoplasia of the first type. This condition is characterized by the fact that a person has the simultaneous presence of endocrine adenomatosis and peptic ulcers of the small intestine.
Zollinger-Ellison syndrome
In any case, gastrinoma is a neoplasm that has:
- round or oval shape;
- dark red shade;
- volumes from two millimeters to two centimeters.
The tumor is characterized by slow growth and a benign course. However, in every third patient with a similar diagnosis, the tumor becomes malignant. Most often metastases spread to:
- regional lymph nodes;
- liver and spleen;
- abdominal cavity and mediastinum;
- skin covering.
Causes
To date, the causes of Zollinger-Ellison syndrome are not fully understood. The main factor in the appearance of this disease is the presence of a tumor in the pancreas or duodenum, which constantly and uncontrollably produces gastrin. Approximately twenty-five percent of patients during the syndrome experience damage to the thyroid gland, as well as the adrenal glands and pituitary gland of the brain.
Zollinger syndrome is sometimes associated with hyperplasia of cells that produce gastrin in the antrum of the stomach. The secretion of the hormone is regulated by the release of hydrochloric acid, but when it is produced by the emerging neoplasm, this process is not controlled, which leads to hypergastrinemia of the organ in which the oncological process has developed.
Forecast
The outcome of Zollinger-Ellison syndrome is somewhat better than the prognosis for other cancers of the digestive organs. This fact is explained by the relatively slow growth rate of gastrinoma. Even when metastases are detected in the liver, the 5-year survival rate is 50-80%, and after radical surgical interventions this figure reaches 70-80%. The death of a patient with Zollinger-Ellison syndrome can be provoked by severe ulcerative complications of this disease.
Classification
In Zollinger syndrome, gastrinoma tumors are classified depending on how many tumors have formed:
- single (solitary) - found in approximately 70% of patients with this disease;
- multiple - diagnosed in 25% of cases of gastrin-producing tumors.
Depending on the area of localization, the following types of gastrinomas are distinguished:
- gastroproducing tumor of the pancreas is the most common tumor affecting the body, head and tail of this organ;
- gastrinoma of the duodenum - a tumor affecting the duodenum;
- gastrinoma of the stomach.
In rare cases, the location of gastrinoma may be the liver or spleen.
In order to seek medical help in a timely manner, it is worth familiarizing yourself with the possible symptoms of this disease in advance.
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Symptoms
When Zollinger Ellison syndrome occurs, the symptoms are nonspecific, which can make further diagnosis difficult. Often, with this syndrome, identified diseases of the duodenum and stomach have atypical foci of ulceration that do not respond to standard methods of therapy. The clinical picture consists of the following main signs:
- Pain syndrome in the upper abdomen that occurs regardless of whether a person has eaten or not. This symptom is more often observed in male patients.
- Disturbances in the act of defecation, with feces of liquid consistency observed. It is usually a single manifestation of gastrinoma, which occurs more often in women.
- Burning and discomfort in the chest area.
- Belching with an unpleasant liquid odor and attacks of heartburn;
- Gastrointestinal bleeding is detected during an instrumental examination of the patient.
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Sometimes there are other signs indicating the presence of gastrinoma:
- nausea with vomiting;
- pale skin color;
- reduction in total body weight;
- surface deformations of teeth;
- the formation of structures that narrow the esophagus.
When Zolligner syndrome occurs in children, the symptoms are more intense than in adult patients.
Diagnostic methods
It is difficult to accurately diagnose Zollinger syndrome, since the clinical picture of the disease is similar to an ordinary lesion of the stomach or duodenum with an ulcer. To make an accurate diagnosis, it is necessary to conduct a complex of various research methods. A preliminary consultation and examination is carried out by a specialist gastroenterologist, during which the doctor examines the patient, palpates the anterior wall of the abdominal cavity, studies his medical history and draws up a preliminary anamnesis. Diagnostics in gastroenterology involves the following laboratory tests:
- general blood test - allows you to identify possible anemia resulting from bleeding in the gastrointestinal tract;
- biochemical blood test - necessary to study the level of gastrin in the blood, which in this disease can reach 1000 pg/ml or more in the blood;
- a test that determines the amount of stomach acid and other contents produced;
- test with secretin - the substance is administered to the patient on an empty stomach to monitor the level of basal gastrin, which decreases in this syndrome (in normal duodenal ulcerations it decreases).
Patients may be prescribed the following instrumental studies:
- EGDS - with a biopsy to determine the structure of tumor cells and tissues;
- Ultrasound of the abdominal organs;
- MRI and CT of the gastrointestinal tract are prescribed to determine the exact location of the gastrin-producing tumor.
When diagnosing this syndrome, it is necessary to differentiate the disease from other possible diseases with a similar clinical picture.
Treatment
Treatment of Zollinger Ellison syndrome is carried out in a radical way, that is, the doctor completely removes the tumor and performs fiber-optic diaphanoscopy of the duodenum and lateral duodenotomy. Often, before surgery, gastrinoma metastases are detected in distant areas, which causes complete cure in only three percent of patients.
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Conservative methods of treatment include the use of medications, the action of which is aimed at reducing the secretion of salt acid:
- PPI;
- H2 receptor blockers.
These drugs in large quantities can be prescribed to the patient for life.
Based on the location of the tumor, surgical procedures can be performed using different methods:
- lateral duodenotomy with removal of the duodenal mucosa;
- fiber-optic diaphanoscopy of the duodenum;
- proxial selective vagotomy;
- partial or complete resection of the stomach;
- total gastrectomy;
- pyloroplasty.
If surgery is contraindicated, radiation and chemical therapy are performed. The effectiveness of all the above methods for this pathology is low, since ulcerations can recur.
Complications
Possible complications include:
- narrowing of the esophageal lumen;
- bleeding in the gastrointestinal tract and subsequent anemia;
- perforation ulcerations;
- severe decrease in body weight;
- disruption of the heart;
- compression of the bile ducts by the tumor;
- malignancy and further metastasis of the tumor.
The cause of complications is usually the patient’s ignorance of clinical symptoms or inadequate treatment. Therefore, at the first signs indicating Zollinger syndrome, you should immediately undergo a medical examination and further treatment.
Description of treatment tactics
The oncological disease in question is dangerous due to its complications and consequences. Under the influence of negative factors, perforation of the ulcer, narrowing of the lower esophagus, and internal bleeding may occur. The list does not end there; there is still a risk of developing cachexia, cardiac dysfunction, tumor metastasis, and compression of the bile ducts.
To prevent all this, you need to consult a doctor in a timely manner. After a comprehensive examination, he will take measures to minimize the release of hydrochloric acid along with gastric juice. For this purpose, antiulcer drugs belonging to the group of proton pump inhibitors are usually used. The fact is that in the conditions of the described disease, ulcers are very resistant to standard therapy, moreover, they are prone to complications. Sometimes situations arise in which the patient's life is at risk. If conservative treatment does not produce results, the doctor decides on the need for surgical intervention. During the procedure, the tumor is completely removed.
Important! To prevent cancer or to prevent it from getting worse, you should monitor your diet and regularly undergo examination by a gastroenterologist. Do not neglect the elimination of bad habits, psycho-emotional stress
Prevention and prognosis
To prevent the occurrence and recurrence of gastrinomas, you should follow some simple rules:
- give up bad habits, in particular smoking and alcohol abuse;
- adhere to a healthy diet and advice from a nutritionist;
- try to avoid chronic stress;
- treat diseases of the gastrointestinal tract and endocrine system in a timely manner;
- undergo regular medical examination.
The prognosis for patients with this syndrome is often favorable, since the tumors are characterized by slow development. Even in the case of metastasis, five-year survival is observed in approximately 50-80% of patients. After radical surgical procedures, the probability of relapse is no more than 30%.
Preventive actions
There is no specific prevention for the development of this syndrome. To minimize the likelihood of developing the disease, you need to adhere to the following recommendations:
Follow healthy eating rules
It is important to limit the amount of fatty, spicy, fried foods. Avoid consuming large amounts of smoked meats, coffee and carbonated drinks.
Be promptly examined by a gastroenterologist, including endoscopic examination. During this diagnostic procedure, the doctor examines the internal surface of the digestive organs. It is advisable to undergo such an examination annually. To refuse from bad habits. You should not smoke or drink a lot of alcohol. Avoid stressful situations.
