Used for Zollinger Ellison syndrome. Zollinger Ellison syndrome symptoms and treatment. Description of treatment tactics

With Zollinger-Ellison syndrome, general symptoms are observed that are characteristic of a common peptic ulcer of the stomach and/or duodenum.

Of the many symptoms, a few of them are the most specific.

• Persistent pain in the upper abdomen. They appear in more than half of patients, more often in men. Occurs after a meal (typical of a gastric ulcer - the formation of defects of varying depths and/or ulcers in the stomach wall) or on an empty stomach/several hours after eating (typical of a duodenal ulcer - the formation of defects of varying depths and/or ulcers in wall of the duodenum).
• Diarrhea (loose stools). Occurs in more than half of patients, more often in women. Often the only symptom. The stool is copious, watery, with a small amount of undigested food and fat.
• A combination of the two above symptoms occurs in more than half of patients.
• Burning behind the sternum, belching, heartburn, which are often mistaken for gastroesophageal reflux disease (regular, often repeated reflux of acidic gastric contents into the esophagus, leading to damage to its lower section).
• Other, less common symptoms:
  • nausea;
  • vomit;
  • gastrointestinal bleeding;
  • weight loss (usually with prolonged loose stools).

Forms

Based on localization (location), there are 3 types of gastrinoma.

• Pancreatic gastrinoma:
  • heads;
  • bodies;
  • tail

• Gastrinoma of the stomach.
• Gastrinoma of the duodenum.

Gastrinoma is most often benign (retains the ability to differentiate (the type of tumor cells is the same as the type of cells of the organ from which it was formed)), but sometimes it can become malignant (degenerate into a malignant (partially or completely lost differentiation of its cells) tumor).

Causes

The primary causes of gastrinoma have not been established. There may be a connection with the transmission of the disease by inheritance from mother to children.

Zollinger-Ellison syndrome may be part of a disease called multiple endocrine neoplasia type 1 (a hereditary combination of endocrine adenomatosis (numerous small tumors without a clear location in the organ) and peptic (arising from excess acid and enzymes) ulcers of the small intestine ( including the duodenum and jejunum)).

Diagnostics

• Analysis of the medical history and complaints (when (how long ago) persistent pain in the upper abdomen, loose stools (diarrhea), burning sensation, pain behind the sternum, belching of sour contents appeared; what the patient associates with the occurrence of symptoms; how the disease progressed).
• Analysis of the patient’s life history (the patient has long-term ulcers that are resistant to antiulcer drugs, other past diseases, bad habits (drinking alcohol, smoking)).
• Analysis of family history (presence of diseases of the gastrointestinal tract (gastrointestinal tract) in relatives).
• Objective examination data:
  • pale skin;
  • bleeding from the gastrointestinal tract;
  • jaundice (jaundiced discoloration of the skin and whites of the eyes due to an increase in the level of bilirubin (one of the bile pigments, a breakdown product of red blood cells) in the blood and tissues). Occurs when the tumor compresses the common bile duct (the vessel through which bile flows from the liver and gallbladder);
  • erosion (surface defect) of teeth.

• Instrumental and laboratory data.
  • Determination of gastrin (a biologically active substance that has a regulating effect on the secretion of gastric juice and the physiological functions of the stomach) in the blood on an empty stomach (on the eve of the study, patients should not take food or medications that alter the production of acid in the stomach).
  • Stomach acid production test (if the acidity (pH) of the stomach is less than 2.0, together with a large volume of measured material (>140 ml), this is a reason to suspect this disease).
  • Test with secretin (a substance that stimulates the pancreas to produce biologically active substances that break down complex food components into simpler ones, which are then absorbed into the body). On an empty stomach, secretin is administered intravenously, and then the level of gastrin in the blood is monitored. An increase in its level >200 pc/ml is a positive test result.
  • Esophagogastroduodenoscopy (a diagnostic procedure during which the doctor examines and evaluates the condition of the inner surface of the esophagus, stomach and duodenum using a special optical instrument (endoscope)) with a mandatory biopsy (taking a fragment of an organ to determine the structure of its tissue and cells).
  • Impedance pH-metry (a procedure based on alternating current measurement of resistance between several electrodes inserted into the esophagus to measure the acidity of the medium).
  • Examination of stomach material and/or respiratory diagnostics for the presence Helicobacter pylori(a bacterium that damages the walls of the stomach and duodenum during its life activity).
  • Ultrasound examination (ultrasound) of the abdominal organs to determine the presence of a tumor of the pancreas, stomach wall or duodenum.
  • Selective abdominal angiography (a low-traumatic diagnostic method, during which, through a puncture of the skin with special instruments under X-ray control, blood is taken from the pancreatic (pancreatic) veins and gastrin is determined in it (a biologically active substance that has a regulating effect on the secretion of gastric juice and on the physiological functions of the stomach )).
  • Computed tomography (CT) to detect a tumor in the pancreas, stomach wall, or duodenum.
  • Magnetic resonance imaging (MRI) is a more accurate diagnostic method than computed tomography. It is carried out to identify a tumor of the pancreas, stomach wall or duodenum.

• Consultations are also possible.

Treatment of Zollinger-Ellison syndrome

Treatment of Zollinger-Ellison syndrome comes down to the following stages of treatment:

• maximum reduction in the secretion of hydrochloric acid and gastric juice with modern antiulcer drugs from the group of proton pump inhibitors;
• in cases of ineffectiveness of conservative (non-surgical) treatment and if a malignant gastrinoma is suspected (a tumor that secretes a biologically active substance that regulates the secretion of gastric juice and affects the physiological functions of the stomach), surgical resection of the tumor (surgical removal of the tumor) is necessary.

Complications and consequences

• Perforation of an ulcer (formation of a hole in the wall of the stomach or intestines) with the development of peritonitis (severe inflammation of the abdominal organs).
• Stricture (significant reduction or narrowing of the lumen) of the lower part of the esophagus due to the constant damaging effect of hydrochloric acid of the stomach.
• The occurrence of gastrointestinal bleeding (bleeding from ulcers of the gastrointestinal tract).
• Significant weight loss up to cachexia (exhaustion).
• Impaired heart function (with persistent diarrhea (loose stools) there is a significant loss of potassium (a trace element responsible for the normal functioning of the heart)).
• Metastasis (formation of tumor foci in other organs, in particular in the liver) of gastrinoma (a tumor that secretes a biologically active substance that regulates the secretion of gastric juice and affects the physiological functions of the stomach).
• Compression of the bile ducts by a tumor, leading to jaundice (jaundice discoloration of the skin and whites of the eyes due to an increase in the level of bilirubin (one of the bile pigments, a breakdown product of red blood cells) in the blood and tissues) and gastrointestinal disorders.

Ulcers in Zollinger-Ellison syndrome are resistant to standard antiulcer therapy and are prone to complications, including life-threatening complications. In this case, surgical removal of the tumor (the cause of the disease) is necessary.

Prevention of Zollinger-Ellison syndrome

There is no specific prevention for Zollinger-Ellison syndrome. Recommended:

• follow the principles of rational nutrition (limit the intake of fried, fatty, spicy and smoked foods, fast food, carbonated drinks, coffee);
• undergo a timely examination by a gastroenterologist, including an endoscopic examination (a diagnostic procedure during which the doctor examines and evaluates the condition of the inner surface of the esophagus, stomach and duodenum using a special optical instrument (endoscope)) - preferably once a year;
• eliminate bad habits (excessive alcohol consumption, smoking).
• avoid psycho-emotional stress.

Additionally

The tumor is most often benign (retains the ability to differentiate (the type of tumor cells is the same as the type of cells of the organ from which it was formed)), but sometimes it can become malignant (degenerate into a malignant (partially or completely lost differentiation of its cells) tumor).

Zollinger-Ellison syndrome– a symptom complex caused by the presence of a gastrin-producing tumor of the pancreas or duodenum, hypersecretion of hydrochloric acid in the stomach and its ulcerogenic effect on the mucous membrane of the gastrointestinal tract. The disease is manifested by symptoms of an ulcer: pain, diarrhea, heartburn, belching, gastrointestinal bleeding. Diagnosis of Zollinger-Ellison syndrome is based on determining the level of basal gastrin, data from endoscopic and X-ray studies, ultrasound, CT, and selective angiography. Treatment for Zollinger-Ellison syndrome may include removal of the gastrinoma, complete gastrectomy, vagotomy, H2 blockers, anticholinergics, proton pump inhibitors, and chemotherapy.

General information

Zollinger-Ellison syndrome is caused by the development of a hormonally active tumor of the pancreas or duodenum that produces excess gastrin, which leads to increased acid formation in the stomach and the development of peptic and duodenal ulcerations.

Gastrin-producing tumors (gastrinomas) belong to the adenomas of endocrine-like cells of the APUD system and are represented by single or multiple dark red nodes of a round (ovoid) shape, dense consistency, and small size (usually from 0.2 to 2 cm). In Zollinger-Ellison syndrome, gastrinomas in the vast majority of cases are localized in the body or tail of the pancreas, about a third in the duodenum or peripancreatic lymph nodes (rarely in the stomach, spleen, liver). Two thirds of gastrinomas are malignant tumors, grow slowly, metastasize mainly to regional and supraclavicular lymph nodes, liver, spleen, mediastinum, peritoneum and skin.

Zollinger-Ellison syndrome is characterized by the presence of multiple ulcers of the upper gastrointestinal tract of atypical localization (for example, in the distal part of the duodenum, in the jejunum), which have a long course and frequent relapses. Zollinger-Ellison syndrome is an uncommon pathology in gastroenterology (about 4 cases per 1 million people), occurring predominantly in males aged 20 to 50 years.

Causes

The main cause of Zollinger-Ellison syndrome is persistent, uncontrolled hypergastrinemia caused by the presence of a gastrin-producing tumor of the pancreas or duodenum. Almost a quarter of patients with Zollinger-Ellison syndrome have multiple endocrine adenomatosis type I, affecting not only the pancreas, but also the pituitary gland, thyroid and parathyroid glands, and adrenal glands.

Sometimes Zollinger-Ellison syndrome can be associated with hyperplasia of gastrin-producing G cells in the antrum. Normally, gastrin secretion by G cells is regulated by a negative feedback mechanism (the inhibitor is the released hydrochloric acid). The production of gastrin by a tumor in Zollinger-Ellison syndrome is not regulated in any way, which leads to uncontrolled hypergastrinemia.

Symptoms of Zollinger-Ellison syndrome

In Zollinger-Ellison syndrome, severe peptic ulcer of the stomach and duodenum of atypical localization, tolerant to antiulcer therapy, may be detected. Clinical symptoms of Zollinger-Ellison syndrome are persistent, intense pain in the upper abdomen, profuse, semi-formed or watery stools containing large amounts of fat (diarrhea and steatorrhea), prolonged heartburn and belching, esophagitis, esophageal strictures. Diarrhea is caused by acidification of the contents of the jejunum, increased peristalsis, the development of inflammation and impaired absorption.

The malignant nature of Zollinger-Ellison syndrome may be indicated by a significant decrease in body weight. With Zollinger-Ellison syndrome, there is a tendency to develop destructive processes and complications (perforation and gastrointestinal bleeding).

Diagnostics

Difficulties in early diagnosis of Zollinger-Ellison syndrome are associated with the presence of symptoms similar to ordinary peptic ulcer disease. Palpation reveals severe pain in the epigastrium, local pain in the area of ​​the ulcer (positive Mendel's symptom).

Differential diagnostic value for Zollinger-Ellison syndrome is the study of the level of basal gastrin in the blood serum and indicators of gastric secretion with functional tests with a standardized food load or intravenous administration of secretin, glucagon, calcium salts. Zollinger-Ellison syndrome, in contrast to ordinary peptic ulcer disease, is characterized by a significant increase in the level of gastrin in the blood (up to 1000 pg/ml or more) and the flow rate of free hydrochloric acid (4-10 times). Specific for Zollinger-Ellison syndrome is a test with secretin, the intravenous administration of which leads to an increase in gastrin levels in most patients (with ordinary duodenal ulcers, its concentration decreases). A stress test with glucagon and calcium gluconate produces a similar effect.

Diagnosis of Zollinger–Ellison syndrome is complemented by instrumental research methods. Suspicion of Zollinger-Ellison syndrome can be caused by the presence of multiple ulcerative lesions and the unusual location of ulcers identified by X-ray of the stomach and FGDS. Ultrasound of the abdominal organs and CT allows one to visualize a tumor of the pancreas; with malignant gastrinoma, a significant enlargement of the liver and the presence of tumor formations in it are also possible. The most informative, but technically more complex method for diagnosing Zollinger-Ellison syndrome is selective abdominal angiography with determination of gastrin levels in the pancreatic veins.

Zollinger-Ellison syndrome is differentiated from difficult to scar and often recurrent ulcers of the upper gastrointestinal tract, celiac disease, tumors of the small intestine, with hypergastrinemia in hyperthyroidism, gastritis, pyloric stenosis, currently indicated only in the absence of a result of conservative treatment and a complicated course of the ulcerative process.

As a conservative treatment practice for Zollinger-Ellison syndrome, drugs are used that reduce the secretion of hydrochloric acid: histamine H2 receptor blockers (ranitidine, famotidine), sometimes in combination with selective m-anticholinergics (platyphylline, pirenzepine), proton pump inhibitors (omeprazole, lansoprazole ). The drugs can be prescribed for life due to the high risk of ulcer recurrence; their doses are higher than in the treatment of conventional peptic ulcer disease and depend on the level of basal secretion of hydrochloric acid. If gastrinoma is malignant and inoperable, chemotherapy (a combination of streptozocin, fluorouracil and doxorubicin) is used.

Forecast

The prognosis for Zollinger–Ellison syndrome is somewhat better than for other malignant tumors and is associated with its rather slow growth: 5-year survival rate even in the presence of liver metastases is 50-80%, after radical surgery - 70-80%. Death may be caused not by the tumor itself, but by complications of severe ulcerative lesions.

For the first time, the combination of a stomach ulcer with a pancreatic tumor, which was manifested by epigastric pain, vomiting large amounts of gastric juice, diarrhea and periodic gastric bleeding, was described in 1901 by V.P. Ruchinsky and F.Ya. Chistyakovich. In 1955, R. M. Zollinder and E. H. Ellison described two observations of persistent progressive gastric ulcer with high gastric hypersecretion, diarrhea in combination with a non-beta cell tumor of the pancreatic islets. These patients were repeatedly operated on for recurrent peptic ulcers. Authors

For the first time, it was suggested that a pancreatic tumor secretes a powerful stimulant of gastric secretion, which leads to the production of large amounts of gastric juice.

It has now been established that a gastrin-producing tumor (gastrinoma) is a paraendocrine tumor that originates from the islets of Langenhars, namely from G-cells producing various types of gastrin, which are normally not found in the pancreas or are not detected at all

in small quantities only in the mucous membrane of the large excretory ducts. This type of neuroendocrine tumor ranks second in frequency among hormonal endocrine tumors of the pancreas, second only to insulinoma.

This tumor occurs with a frequency of 2-4 cases per 1 million population; the ratio of men to women is 3:2. The cause of recurrence of ulcers after gastric surgery is insulinoma in approximately 1% of patients.

In 60% of patients, gastrinomas are localized in the tail or head of the pancreas and in 15-20% in the submucosal layer of the duodenum, the initial part of the jejunum, stomach, liver, and adrenal glands.

There are two forms of the syndrome:

Sporadic – usually occurs after 40 years, in 60–90% of cases the tumor is malignant with a slow growth rate;

A genetically determined disease occurs against the background of Wermer's syndrome (genetically determined type I adenomatosis with damage to the parathyroid glands of the pituitary gland, etc.). This form is often

is multiple in nature and occurs in young people. At the beginning of its development, gastrinoma is benign, but later it tends to undergo malignant transformation. The disease is usually familial in nature.

The sizes of gastrinomas range from 2 mm to 5 cm or more.

Gastrinoma produces gastrin with a molecular weight of 34 big-gastrin, which stimulates the parietal and chief cells of the gastric mucosa and causes their hyperplasia. At the same time, acid-peptic products reach such a level that even an intact mucous membrane cannot resist it.

In most patients, ulcers are localized in the duodenum, less often in the stomach and jejunum, and after gastric surgery in the area of ​​the gastrojejunal anastomosis.

Ulcers in Zollinger-Ellison syndrome are characterized by severity, decreased susceptibility and healing, persistent recurrence and frequent development of complications.

Clinical picture. Patients are bothered by intense epigastric pain that is not amenable to antiulcer therapy. Severe heartburn and sour belching are observed - symptoms of esophagitis are often observed. Vomiting is usually accompanied by the release of large amounts of gastric contents.

30-60% of patients have diarrhea with profuse watery stools and steatorrhea, which is explained by the ingestion of large

volume of gastric juice with a high concentration of hydrochloric acid, as well as increased intestinal peristalsis. In severe cases, diarrhea can lead to dehydration and hypovolemia. Possible weight loss, which is especially typical for malignant gastrinoma.

Palpation of the abdomen reveals pain in the epigastrium or pyloroduodenal region.

Laboratory and instrumental research methods.- All patients have pronounced gastric hypersecretion, especially in

night time (about 2-4 liters are released). - The main diagnostic test is the determination of gastrin in the blood plasma.

In healthy people, the concentration of gastrin in plasma is in the range of 50-100 pg/ml; in gastroduodenal ulcers it also does not exceed 100 pg/ml. The gastrin content in the blood with insulin increases to 1000-2000 pg/ml or more.

Secretin test. The patient is injected intravenously with 1-2 units of secretin per 1 kg of body weight. With insulinoma, gastric production increases and the gastrin content in the blood increases significantly. IN

At the same time, with gastroduodenal ulcers, gastric secretion is inhibited and the gastrin content in the blood decreases.

Test with calcium gluconate. Calcium gluconate is administered intravenously at a dose of 4-5 mg per 1 kg of body weight. With insulinoma, the level of gastrin in the blood increases; with peptic ulcer disease, the increase is insignificant.

Topical diagnosis of the tumor itself is carried out using ultrasound, CT, MMT, endoscopic ultrasound and selective angiography. However, these methods can detect patient tumors only in 40-60% of patients and mainly if the tumor is larger than 0.5 cm.

The most effective method for diagnosing insulinomas is scincigraphy of samostatin receptors with synthetic analogues of samostatin (ocreosan, octreotide) labeled with radioactive In. The reliability of the method reaches 90% and does not depend on the size of the tumor.

Treatment.. In order to suppress acid production of the stomach and prevent recurrence of ulcers, as well as severe complications associated with it, patients are prescribed massive doses of antisecretory therapy. Doses of H2 blockers and proton pump inhibitors should be 4-6 times higher than usual, and the duration of treatment should be increased.

The initial dose of famotidine is 40 mg 4 times a day, the daily dose of ranitidine is up to 1200 mg.

The drug of choice is omeprozole, which is prescribed 60 mg 2 times a day.

The choice of surgical intervention in patients with Zollinger-Ellison syndrome is determined by the primary location of the tumor.

If a single small tumor is detected in the pancreas or stomach, it is enucleated or economically resected.

For large tumors in the pancreas and suspected malignancy, resection of part of the pancreas or pancreaticoduodenectomy with removal of all groups of regional lymph nodes is indicated.

Gastrinoma metastases to the liver are subject to surgical treatment, and in cases where it is impossible to perform surgery, chemotherapy, embolization of hepatic vessels, and octreotide are prescribed. These patients receive maintenance antisecretory therapy with omeprozole for life.

Surgical treatment is not indicated for patients with multiple endocrine neoplasia type I and Zollinger-Ellison syndrome. They are prescribed lifelong use of antisecretory drugs.

Zollinger-Ellison syndrome is diagnosed quite rarely. This pathology can occur at any age. However, it is most often discovered between 20 and 50 years of age. If signs of illness appear, you should contact a gastroenterologist who will make an accurate diagnosis. Therapy most often involves the use of drugs that reduce the production of gastric juice and promote healing of ulcers. In any case, a doctor should tell you about the symptoms and treatment of Zollinger-Ellison syndrome.

Many patients are interested in what Zollinger-Ellison syndrome is. The description of the disease is as follows: this term refers to a condition that is provoked by the presence of a tumor lesion of the pancreas, which synthesizes gastrin. This formation can be localized in the wall of the stomach. Damage to the jejunum or duodenum is often observed.

With an increase in gastrin content in the blood, the synthesis of gastric juice, enzymes and hydrochloric acid is activated. As a result, many ulcerative lesions of varying depths are formed.

The pathology can develop in any person, but men are more susceptible to it.

Doctors note the following types of disease:

1. Solitary formations - such tumors are diagnosed in 70% of cases. They are localized in the pancreas.
2. Multiple tumors – observed in 25% of cases. In appearance they are endocrine neoplasias. Foci of formations are present in the pancreas, adenohypophysis, and parathyroid glands. They can also be detected in the fatty tissue of the abdominal tissue.
3. Hypergastrinemia is extremely rare. In this case, lipoma, formations in the adrenal glands, and angiomyolipoma of the kidneys appear.

Causes

So far, scientists have not been able to accurately determine the factors that provoke the appearance of this syndrome. The main cause of the disease is considered to be constant and uncontrolled release of gastrin. This is due to the appearance of a tumor.

In approximately 25% of cases, people are diagnosed with multiple adenomatosis of the first type, which is endocrine in nature. In this case, tissue damage is observed not only to the pancreas, but also to other types of glands - the thyroid, pituitary gland, parathyroid.

In some patients, the occurrence of the syndrome is caused by hyperplasia of gastrin-producing G cells. They are localized in the antral zone of the stomach. There is also a theory about the hereditary nature of the pathology. In this case, the genetic tendency is passed on from parents to children.

Symptoms

With Zollinger-Ellison syndrome, common symptoms are observed that accompany peptic ulcer of the digestive organs. Among the large number of manifestations, several characteristic symptoms can be identified. These include the following:

1. Constant discomfort in the upper abdomen. This sign is present in more than 50% of cases. Most often it is observed in men. Discomfort appears after eating, which is typical for a stomach ulcer, or on an empty stomach - this indicates damage to the duodenum.
2. Diarrhea. This symptom also appears in more than half of the cases, but it is more typical for women. Often diarrhea is the only sign of a disorder. The stool is watery and contains some fat and undigested food.
3. A combination of abdominal pain and diarrhea. This condition is observed in more than 50% of cases.
4. Heartburn, burning in the chest area, belching. These signs are often mistaken for gastroesophageal reflux disease. This term refers to the systematic entry of acidic stomach contents into the esophagus, which causes damage to the lower part of the organ.

More rare manifestations of this syndrome include the following:

• Nausea;
• vomit;
• weight loss – most often observed with prolonged diarrhea;
• bleeding in the digestive organs.

Diagnostics

If such symptoms appear, consultation with a specialist is required. Diagnosis of Zollinger-Ellison syndrome should be carried out by a gastroenterologist. During the initial examination, the doctor will take an anamnesis. A physical examination is considered a mandatory part of the examination. Based on its results, the specialist will identify pain in the epigastric area and enlargement of the liver.

In addition, the doctor will prescribe additional examinations, including the following:

• A biochemical blood test to determine the gastrin content - it must be taken on an empty stomach;
• scintigraphy with octreotide;
• secretin test;
• radiography;
• CT scan;
• assessment of basal acid production;
• abdominal selective angiography;
• Magnetic resonance imaging.

Treatment

If a pathology is detected, the patient must be hospitalized in the gastroenterological or surgical department. If a malignant tumor is detected, the person needs to be treated in an oncology clinic.

The goal of drug treatment for Zollinger-Ellison syndrome is to reduce acidity, prevent new ulcerative lesions and heal existing ones. For this purpose, the following medications may be prescribed:

• H2-histamine blockers - these include Famotidine and Ranitidine;
• proton pump inhibitors - this category includes Rabeprazole, Omeprazole;
• M-anticholinergics - the doctor may prescribe Pirenzepine, platyphylline hydrochloride;
• somatostatin analog – Octreotide can be used.

On a note. To slow down the development of the formation or reduce its size, cytostatics can be used. This category includes drugs such as Streptozocin, Doxorubicin, etc.

In some cases, it is not possible to do without surgery. If the gastrinoma has not metastasized, a minimally invasive procedure is performed to remove it. In more complex situations, a total gastrectomy or removal of the stomach is indicated.

Possible complications

If therapy is not started in time, the disease can lead to dangerous consequences. These include:

1. Ulcer perforation – involves the appearance of a hole in the stomach or intestines. This often leads to the development of peritonitis, which is a complex lesion of the abdominal organs.
2. Stricture of the lower part of the esophagus is a significant narrowing of the lumen. This is due to constant exposure to hydrochloric acid in the stomach.
3. Significant weight loss – in difficult cases, exhaustion of the body may occur.
4. Impaired cardiac function - with constant diarrhea, there is a significant loss of potassium. It is this element that is responsible for the normal operation of this body.
5. Metastasis of gastrinoma is the appearance of tumors in other organs. The liver is most often affected.
6. Compression of the bile ducts by the formation causes jaundice and digestive disorders.
7. Digestive bleeding.

On a note. Ulcerative defects in this syndrome are resistant to standard therapy and are prone to complications. They can pose a real health hazard. In such a situation, surgical removal of the formation is required.

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:

1. Follow the rules of healthy eating. It is important to limit the amount of fatty, spicy, fried foods. You should not consume large amounts of smoked meats, coffee and carbonated drinks.
2. 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.
3. To refuse from bad habits. You should not smoke or drink a lot of alcohol.
4. Avoid stressful situations.

Forecast

The prognosis for this pathology is slightly better than for other malignant tumors. This is due to the slow development of the tumor.

Even if metastases are detected in the liver, the five-year survival rate is 50-80%. After major operations, this figure is at the level of 70-80%. Death is usually caused not by the tumor itself, but by the consequences of complex ulcerative defects.

Zollinger-Ellison syndrome is a fairly serious pathology that can cause dangerous complications. To minimize the negative consequences of violations, you should consult a doctor in a timely manner and strictly follow his prescriptions.

Zollinger-Ellison syndrome (ZES) is a fairly rare disease during which one or more tumors (gastrinomas) appear in the duodenum or pancreas. As a result of the development of pancreatic gastrinoma, the acidity of gastric juice increases. This causes the formation and development of peptic ulcers, diarrhea, etc.

Causes of the syndrome

Experts still don’t know exactly what causes Zollinger-Ellison syndrome. However, the sequence of progression of this disease is clear to them. When Zollinger-Ellison disease appears in the area of ​​the duodenum, pancreas, and lymph nodes near the liver, a tumor appears.

The pancreas is located below and behind the stomach. It serves to provide enzymes to the digestive organs and produces hormones that affect the maintenance of a normal amount of glucose in the blood.

The cells of the neoplasm, which appeared as a result of PZE, produce an excess amount of gastrin, as a result of which too much hydrochloric acid is produced in the stomach. Excessive amounts of hydrochloric acid in turn lead to the formation of peptic ulcers and other negative effects.

In addition to the fact that the tumor affects not only the synthesis of hydrochloric acid, it can also become malignant. Despite the fact that the growth of these formations occurs quite slowly, cancer can affect nearby organs and systems, especially the lymph nodes, which are located next to the liver.

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Diagnosis of the disease

The diagnosis by a gastroenterologist is based on the following examinations:

1. The doctor should evaluate the patient's medical history to find out about gastrointestinal diseases that have been diagnosed previously, including in family members.
2. Examination of blood samples to determine the amount of gastrin in it. A high level of gastrin may indicate a possible tumor of the pancreas, duodenum, or other pathology. So, in the case of using antacids or low stomach acid production, the gastrin content may be excessive. To take a test to determine the exact level of gastrin in the blood, you must first stop using antacids and not eat anything. Since the amount of gastrin content can change periodically, tests are taken several times after a certain time.
3. Your healthcare provider may perform a test that involves secretin stimulation. This test takes measurements before and after the administration of secretin. If, after the administration of the latter, the gastrin content becomes even higher, then this is a clear sign of the presence of Zollinger-Ellison disease in the patient.
4. Gastroduodenoscopy. During this test, the doctor looks for ulcers using an endoscope equipped with a camera and a light source inserted into the duodenum and stomach. This procedure detects gastrin-producing tumors by taking tissue samples from the duodenum. To get the most out of the test, you should not eat after midnight on the day of the test.
5. Imaging studies. During this study, a specialist may use somatostatin receptor scintigraphy. This method makes it possible to detect tumors thanks to radioactively labeled atoms. Useful imaging tests include MRI, CT, and ultrasound.
6. Endoscopic ultrasound. In this study, a specialist uses an endoscope equipped with an ultrasound sensor to examine the pancreas, duodenum and stomach. The examination allows you to get a good look at possible tumors up close and take a tissue sample for more detailed analysis.

Treatment Options

Treatment of Zollinger-Ellison syndrome can be carried out in 2 ways - surgical and therapeutic.

The therapeutic method is used when the tumor is not malignant. In this case, the patient is prescribed medications that significantly reduce the acidity of gastric juice. These same drugs will also have a beneficial effect on ulcerative formations. The most commonly prescribed drugs are Esomeprazole, Omeprazole, Lansoprazole and their other analogues.

In the case of a critical condition of the patient, when the disease is already advanced and ulcers have begun to develop on the walls of the organs, it is no longer possible to do without surgical intervention.

To remove tumors that appear as a result of Zollinger-Ellison syndrome, a highly qualified operating surgeon is required. This is because gastrinomas are difficult to see and tend to be small in size. When there is only one tumor, its removal can be done quite easily, but with a large number of gastrinomas or with liver damage, surgical intervention is sometimes impossible. If there are multiple tumors, specialists may suggest removing only the largest of them.

• try to remove the tumor using radio wave ablation (use of high temperatures) or embolization (deprivation of blood supply to the malignant neoplasm);
• removal of the largest part of the liver;
• to relieve symptoms, injecting drugs directly into the tumor itself;
• liver transplant;
• use of chemotherapy.

Preventive measures

Since concomitant diseases in the occurrence of Zollinger-Ellison disease are pathologies of the digestive organs, when considering preventive measures, one should mainly adhere to the following recommendations:

• undergo regular examination by a specialist;
• strictly observe and perform all actions related to therapy that were prescribed by the attending physician;
• avoid stress;
• reduce physical activity;
• consult and follow all recommendations of a nutritionist.

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It is believed that Zollinger-Ellison disease is a rather dangerous and serious pathology. And if you do not take into account the symptoms of the disease and refuse the need to perform an operation, then in this case trophic ulcers of the duodenum, stomach, pancreas, and even hepatitis, exhaustion, and gastric bleeding may occur. But timely measures can not only prevent the development of the syndrome, but also avoid the spread of metastases if the neoplasm is malignant.

Early diagnosis of the disease and the presence of only 1 tumor increase the likelihood of a favorable prognosis. In case of late diagnosis of the disease and its treatment, in 60% of cases, patients develop pancreatic cancer.