Tumor diseases are diagnosed in patients of different sex and age, and they can cause a variety of health disorders - depending on their localization, structure, size, nature and individual features person. Unfortunately, doctors have not yet been able to determine the exact factors that provoke pathological growth body tissues and/or their malignancy. So the reason for the formation of a tumor of the pancreas or duodenum is also not known. Such a formation can cause Zollinger-Ellison syndrome, the symptoms and treatment of which we will consider, we will also talk about what the diagnosis of such an ailment should be.

Zollinger-Ellison syndrome is a whole complex of symptoms that occurs due to the development of a hormonally active tumor formation in the pancreas or in duodenum. Such a tumor synthesizes an excessive amount of gastrin, which causes increased acid production in the stomach, which leads to ulcers in the stomach and duodenum.

Symptoms of Zollinger-Ellison Syndrome

With Zollinger-Ellison syndrome, doctors detect a severe peptic ulcer of the stomach and duodenum, which is characterized by atypical localization and is resistant to antiulcer treatment.

The classic manifestations of Zollinger-Ellison syndrome are the appearance of persistent intense pain in the upper abdomen, as well as the occurrence of semi-formed or watery stools, which contain quite a lot of fat (in this case, they talk about a combination of diarrhea and steatorrhea). Patients complain of prolonged (frequent) sour belching and heartburn, they have esophageal stricture, symptoms of esophagitis. They may also be disturbed by a burning sensation behind the sternum, a feeling of nausea (sometimes turning into vomiting).

Diarrhea in this pathology is explained by acidification of the contents of the jejunum, as well as the activation of peristalsis, the occurrence of an inflammatory process and impaired absorption. In some cases, it becomes the only manifestation of the Zollinger-Ellison syndrome.

The malignant nature of such symptoms can be judged by a significant decrease in body weight.

It should be noted that patients with Zollinger-Ellison syndrome become prone to destructive processes, they often develop complications (perforations and digestive bleeding).

How is Zollinger-Ellison syndrome detected, what does the diagnosis give?

It is not easy to identify the Zollinger-Ellison symptom, since its manifestations on initial stage development is completely similar to common peptic ulcer. The doctor collects a detailed anamnesis, conducts an objective examination. Studies are being conducted on the level of gastrin, on the production of acid by the stomach and the so-called secretin test. In addition, esophagogastroduodenoscopia and impedance pH-metry are often performed. The material of the stomach is examined for the presence of Helicobacter pylori.

Among the classic studies that allow the diagnosis of Zollinger-Ellison syndrome are ultrasound of the peritoneum, selective abdominal angiography, computed tomography and MRI.

How is Zollinger-Ellison syndrome corrected, what is its effective treatment?

Treatment of Zollinger-Ellison syndrome can be either conservative or surgical. An operation to completely eliminate a gastrinoma is considered radical, but such manipulation is possible only in ten percent of patients. Unfortunately, by the time the tumor is detected, most patients have metastases in various organs. In addition, there are situations when the tumor cannot be detected during laparotomy due to its small size.

That is why doctors previously resorted to total gastrectomy (radical removal of the entire stomach). Now, thanks to the active use of histamine H2 receptor blockers in therapeutic practice, the number of indications for such surgery has decreased. Total gastrectomy is carried out if the above drugs do not give a positive effect, and also if there is a complicated course of ulcers.

Quite often, doctors spend conservative treatment Zollinger-Ellison syndrome. In this case, drugs are used that can reduce the production of of hydrochloric acid. The drugs of choice are the above-mentioned histamine H2-receptor blockers, represented by Ranitidine and Famotidine. In some cases, they are combined with selective m-anticholinergics, for example, with Platifillin or Pirenzepine. In addition, proton pump inhibitors, namely omeprazole or lansoprazole, can be used.

Drug treatment quite often it is lifelong, since the disease is prone to relapse. At the same time, the dosage of drugs is an order of magnitude higher than in the treatment of ordinary ulcerative lesions, it depends on the detected level of basal secretion of hydrochloric acid.

In the event that the gastrinoma is malignant and inoperable, chemotherapy is carried out with a combination of Streptozocin, Fluorouracil, and Doxorubicin (instructions for the use of each drug must be studied personally from the official annotation enclosed in the package before use!).

It should be noted that the prognosis for a patient with Zollinger-Ellison syndrome is slightly better than with other oncological lesions. This is due to the rather slow growth of the tumor: a five-year survival rate can reach fifty to eighty percent, even if the patient has liver metastases.

Often death occurs not because of the tumor itself, but because of the complications of severe ulcers.

Folk recipes

Funds traditional medicine may contribute to the healing of ulcerative lesions in Zollinger-Ellison syndrome, but they can be used only after consultation with your doctor.

So wonderful anti-inflammatory, bactericidal and healing qualities are characterized by the medicinal plant Kalanchoe Peritoe. It can be taken on its own by simply chewing the freshly picked leaves of the plant. Of course, before that, they need to be thoroughly washed. Eat such raw materials immediately before meals.

You can also take Kalanchoe juice for medicinal purposes. It should be drunk one or two teaspoons a quarter of an hour before a meal. Repeat the reception three times a day. The recommended duration of such therapy is one month.

Zollinger-Ellison syndrome is a complex clinical signs caused due to the development of a hormonally active neoplasm in the pancreas or duodenum. This is a cancer, the development of which leads to the production of gastrin, it is characterized by the presence of multiple ulcers on the mucosa of the pancreas or duodenum 12. With this syndrome, patients experience:

pain;
diarrhea;
heartburn;
gastrointestinal bleeding;
violation of the act of defecation;
constant burps.

Often, the pathology proceeds with non-specific symptoms, which greatly complicates the diagnosis of the disease itself. This pathology can be easily confused with the occurrence of an ordinary ulcerative process in the stomach or duodenum. Treatment this disease carried out in a complex manner, combining surgical manipulations with conservative therapy, through the use special preparations. Without surgery, you can only slightly improve the patient's condition.

What is Zollinger Ellison Syndrome

Zollinger's syndrome was first identified in 1955, when two patients were found to develop peptic ulcers in the upper sections. digestive tract. This condition is caused by developing hormonally active tumor in the area of the pancreas - gastrinoma. The nature of the gastrinoma is very aggressive, it produces an excessive amount of gastrin, resulting in increased production of gastric acid and the development of stomach ulcers.

Gastrinomas belong to the group of adenomas with endocrine-like cells. A significant part (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.

Outwardly, neoplasms are single or multiple dark red nodes with a dense texture and rounded shape, more often they do not have big size and reach only 0.2-2 cm. 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 site of localization is the peripancreatic lymph nodes or small intestine. Sometimes it is possible to form neoplasms in the spleen, liver tissues and stomach. The development of such a pathology is rare, only four cases per one million population. Most often, men from twenty to fifty years of age are exposed to the disease.

Causes

To date, the causes of Zollinger-Ellison syndrome have not been fully understood. The main factor in the appearance of this disease is the presence or duodenal ulcer, which constantly and uncontrollably produces gastrin. Approximately twenty-five percent of patients during the syndrome have damage to the thyroid gland, as well as the adrenal and pituitary glands of the brain.

Sometimes Zollinger's syndrome is associated with hyperplasia of cells that produce gastrin in antrum stomach. The secretion of the hormone is regulated by the release of hydrochloric acid, but when it is produced by the resulting neoplasm, this process is not controlled, which leads to hypergastrinemia of the organ in which the oncological process has developed.

Classification

In Zollinger's syndrome, gastrinoma tumors are classified according to how many neoplasms have formed:

solitary (solitary) - occur 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 gastrinoma are distinguished:

gastroproducing tumor of the pancreas - the most common tumor affecting the body, head and tail of this organ;
duodenal gastrinoma - a tumor affecting the duodenum;
gastrinoma of the stomach.

In rare cases, the liver or spleen can serve as the site of gastrinoma localization.

To apply in a timely manner medical care It is worth familiarizing yourself with the possible symptoms of this disease in advance.

Symptoms

When Zollinger-Ellison syndrome occurs, the symptoms are non-specific, which can make further diagnosis difficult. Often, with this syndrome, the identified diseases of the duodenum and stomach have atypical foci of ulceration that are not amenable to standard methods of therapy. The clinical picture consists of the following main features:

Pain in the upper abdomen, which occurs regardless of whether a person has taken food or not. This symptom is more common in male patients.
Violations of the act of defecation, while observed feces of a liquid consistency. It is usually a single manifestation of gastrinoma, which occurs more often in women.
Burning and discomfort in the retrosternal region.
Belching with an unpleasant liquid odor and bouts of heartburn;
Gastrointestinal bleeding - found when instrumental examination sick.

Sometimes there are other signs indicating the presence of a gastrinoma:

nausea with gusts of vomiting;
pale skin color;
decrease in total body weight;
superficial deformities of teeth;
formation of structures narrowing the esophagus.

When Zolligner's syndrome occurs in children, the symptoms are more intense than in adult patients.

Diagnostic methods

An accurate diagnosis of Zollinger's syndrome is difficult because clinical picture disease converges with an ordinary lesion of the stomach or duodenal ulcer. For an accurate diagnosis, it is necessary to carry out a complex various methods research. Preliminary consultation and research is carried out by a specialist gastroenterologist, during which the doctor examines the patient, performs palpation of the anterior wall abdominal cavity, studies his medical history and makes a preliminary anamnesis. Diagnostics in gastroenterology includes such laboratory research:

complete blood count - allows you to identify possible anemia resulting from bleeding in the gastrointestinal tract;
a biochemical blood test - is 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 produced stomach acid and other contents;
test with secretin - the substance is administered to the patient on an empty stomach to control the level of basal gastrin, which decreases with this syndrome (it decreases with ordinary duodenal ulceration).

Patients may be assigned the following instrumental studies:

EFGDS - with a biopsy to determine the structure of cells and tumor tissues;
Ultrasound of the abdominal organs;
MRI and CT of the gastrointestinal tract - is prescribed to establish the exact localization of the tumor that produces gastrin.

When diagnosing this syndrome, it is imperative 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 found in distant areas, which causes complete cure only three percent of patients.

Conservative methods of treatment include the use of drugs, the action of which is aimed at lowering the secretion of acid salts:

H2 receptor blockers.

These drugs in large quantities can be prescribed to the patient for life.

Based on the location of the tumor, it is possible to perform surgical procedures different methods:

lateral duodenotomy with removal of the duodenal mucosa;
fiber optic diaphanoscopy of the duodenum;
proximal selective vagotomy;
partial or complete resection of the stomach;
total gastrectomy;
pyloroplasty.

If the operation is contraindicated, then radiation and chemical therapy are performed. The effectiveness of all the above methods for such a pathology is small, since ulcerations can recur.

Complications

To possible complications can be attributed to:

narrowing of the esophageal lumen;
bleeding in the gastrointestinal tract and subsequent anemia;
perforation ulceration;
a strong decrease in body weight;
disruption of the heart;
tumor compression of the bile ducts;
malignancy and further metastasis of the neoplasm.

The cause of complications is usually ignoring the patient's clinical symptoms or inadequate treatment. Therefore, at the first signs indicating Zollinger's syndrome, one should pass without delay medical examination and further treatment.

Prevention and prognosis

To prevent the occurrence and recurrence of gastrinoma, you should follow some simple rules:

give up bad habits in particular smoking and alcohol abuse;
stick to healthy eating and advice from a dietitian;
try to avoid chronic stress;
timely treat diseases of the gastrointestinal tract and the endocrine system;
undergo regular medical examinations.

The prognosis for patients with this syndrome is often favorable, since 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 manipulations, the probability of recurrence is no more than 30%.

Zollinger-Ellison syndrome (pancreatic adenoma ulcerogenic, gastrinoma) is a tumor of the islet apparatus of the pancreas, characterized by the occurrence of peptic ulcers of the duodenum and stomach, which cannot be treated and is accompanied by persistent diarrhea.

The true incidence of Zollinger-Ellison syndrome has not been established. According to some reports, it occurs in 0.1-1% of patients with peptic ulcer of the stomach and duodenum. Most often, the initial manifestations of this disease occur at the age of 30-60 years.

Causes and development of the disease

The syndrome is the result of the development of a tumor (gastrinoma) in the head or tail of the pancreas (85% of cases). In 15% of cases, the tumor is localized in the stomach or in the duodenum. Cases of multiple endocrine adenomatosis (multiple endocrine neoplasia) have been described.

About two-thirds of gastrinomas are malignant; in a third of patients, metastases are detected at the first visit to the doctor. Usually, malignant gastrinomas grow slowly, but sometimes they progress rapidly and metastasize early. Most often, gastrinomas metastasize to regional lymph nodes and the liver, as well as to the peritoneum, spleen, bones, skin, and mediastinum.

The development of treatment-resistant peptic ulcers is the main manifestation of the disease in almost all patients. Ulceration is due to the production of gastrin by the tumor, and, accordingly, increased secretion gastric juice, i.e. hydrochloric acid and enzymes. In the vast majority of patients, a single ulcer is localized in the duodenum and stomach (75%), as well as in the jejunum. There are also multiple ulcers of the stomach, duodenum and jejunum.

Complaints and symptoms

Clinical manifestations of the disease are pains in the upper abdomen, which have the same patterns in relation to food intake as in the case of a normal duodenal ulcer and stomach, but unlike them, they are very stubborn, very intense and do not respond to antiulcer therapy.

Characterized by persistent heartburn and belching sour. An important sign are diarrhea caused by the ingestion of a large amount of hydrochloric acid into the small intestine and, as a result, an increase in the motility of the small intestine and a slowdown in absorption. Stools are profuse, watery, with a lot of fat. Perhaps a significant decrease in body weight, which is typical for malignant gastrinemia.

Ulcers of the stomach and duodenum in Zollinger-Ellison syndrome do not heal even with prolonged complex therapy. Many patients experience esophagitis, sometimes even with the formation of a narrowing (stricture) of the esophagus. When probing, severe pain is determined in the upper abdomen, and the area of the projection of the lower part of the stomach.

In the case of a malignant course of the disease, tumor formations in the liver and its significant increase are possible.

Diagnostics

X-ray and endoscopic examination reveal an ulcer that does not differ from that in normal peptic ulcer duodenum. The main laboratory criterion for Zollinger-Ellison syndrome is hypergastrinemia (the content of gastrin in the blood is increased to 1000 pg / ml or more, while in ordinary peptic ulcer it does not exceed upper bound norms - 100 pg / ml).

The detection of the tumor itself is performed using ultrasound, computed tomography, selective abdominal angiography. The method of selective abdominal angiography with blood sampling from pancreatic veins and the determination of gastrin in it has the greatest information content.

However, it is difficult to establish the localization of gastrinoma. In about half of patients with clinically and laboratory confirmed Zollinger-Ellison syndrome, a tumor cannot be found during surgery. Selective angiography can detect gastrinoma in 50%, CT in 30%, and ultrasound in 20% of cases.

Treatment

The main method of treatment is surgical removal of a potentially malignant tumor.

Conservative treatment aims to significantly reduce gastric secretion and thereby achieve healing of the peptic ulcer. To do this, proton pump inhibitors are prescribed with a 2-3-fold increase in dosage: Lansoprazole (per os 30-160) or Omeprazole (per os 20-120 mg / day).

Zollinger-Ellison Syndrome

What is Zollinger-Ellison syndrome (general information)

Zollinger-Ellison syndrome is rare disease characterized by the development of a tumor, called a gastrinoma, found in the pancreas and/or duodenum. Gastronomers secrete excessive levels of gastrin, a hormone that stimulates production.

Normally, the body itself releases a small amount of gastrin after eating, which causes the stomach to produce stomach acid, which helps break down food and liquid in the stomach. However, excess acid causes peptic ulcer formation elsewhere in the upper intestine.

Although anyone can get Zollinger-Ellison syndrome, the disease is more common in men between the ages of 30 and 50. The prevalence of the disease occurs in about 10 people per million population.

Medical researchers are still investigating the exact cause of Zollinger-Ellison syndrome. About 25-30 percent of gastrinoma cases are caused by hereditary genetic disease called multiple endocrine neoplasia type 1 (MEN1). MEN1 causes hormone-releasing tumors in the endocrine glands and duodenum. Symptoms of MEN1 include elevated blood hormone levels, kidney stones, diabetes, muscle weakness, weakened bones, and fractures.

Symptoms of Zollinger-Ellison Syndrome

The signs and symptoms of Zollinger-Ellison syndrome are similar peptic ulcer symptoms. A dull or burning pain felt somewhere between the navel and the middle chest is the most common symptom of a peptic ulcer.

Other symptoms of Zollinger-Ellison syndrome include:

mild or severe abdominal pain;
steatorrhea (increased amount of fat in the stool);
bloating;
nausea;
vomiting;
weight loss;

Some people with Zollinger-Ellison syndrome have only diarrhea with no other symptoms. Others develop (GER), which occurs when the contents of the stomach back up into the esophagus.

It is recommended that you consult your doctor if you have persistent abdominal or supra-abdominal pain accompanied by recurring episodes of diarrhea, vomiting, and nausea. It is important not to neglect these disorders, because a disease such as Zollinger-Ellison syndrome, if diagnosed late, is difficult to successfully treat.

Complications

The recurrence of peptic ulcer due to the constant presence of acid secretions in the stomach can cause gastrointestinalbleeding and perforation. In addition, since gastrinomas are malignant tumors, there is a risk that they may distribute metastases, especially in .

Metastases are cancer cells that have moved from their original location to another location, contaminating the lymph nodes and/or other organs in the body.

Causes

In most people, Zollinger-Ellison syndrome appears to occur spontaneously for unknown reasons (sporadically). However, in about 25-30 percent of affected individuals, the syndrome occurs in association with a genetic disorder known as multiple endocrine neoplasia type 1 (MEN1). In most patients, MEN1 is inherited as an autosomal dominant genetic disorder.

Dominant genetic disorders occur when only one copy of a broken gene is passed on to a person to cause a particular disease. The non-working gene can be inherited from either parent, or it can be the result of a mutation (change) in the gene in the affected person. The risk of passing the broken gene from an affected parent to offspring is 50% for each pregnancy.

MEN-1 is caused by changes (mutations) in the gene MEN1. Gene MEN1 regulates the production of a protein (called "menin") that appears to play a role in preventing tumor development (tumor suppressor).

Diagnostics

The diagnosis of Zollinger-Ellison syndrome is based on a thorough clinical evaluation, a detailed patient history, and specific tests, including certain laboratory tests and advanced imaging techniques. Zollinger-Ellison syndrome can be caused by a variety of factors, including the development of frequent or multiple peptic ulcers that are resistant to certain standard ulcer treatments and/or that occur in unusual locations (eg, in the jejunum).

In individuals with suspected Zollinger-Ellison syndrome diagnostic studies may include a blood test to check for elevated levels gastrin and evaluation of gastric juice samples to detect elevated levels of acid. Some patients may also have additional laboratory tests to confirm the syndrome. Such tests may include measuring the level of gastrin in the liquid part of the blood (serum) before and after intravenous infusion calcium; injection of the digestive hormone secretin. Additional laboratory tests may also be done to confirm or rule out MEN1.

Treatment of Zollinger-Ellison Syndrome

In the case of Zollinger-Ellison syndrome, the best therapeutic solution is surgical removal of the gastrin. However, the conditions for the implementation of this type of operation are not always met.

An alternative solution is to treat the disease as a stomach ulcer, in which patients take proton pump inhibitors (PPIs) and H2 antagonists (anti-H2). The use of these drugs acts only on the symptoms, removing them, but does not regress the tumor and does not protect the patient from the possible spread of metastases.

Liver metastases can be removed surgically only if the malignant cells that have reached the liver are concentrated in a limited space and are not deep inside.

Surgical intervention

Gastrinomas are surgically removed with laparoscopic or laparotomic surgery, provided that they are single and well localized. If, on the other hand, gastrinomas are multiple and scattered over several sites, or if they are associated with MEN1, surgical intervention is an impractical solution.

Alternatively, but only in certain situations, you can try to solve the problem in one of the following ways:

Surgical removal of only the largest gastrinoma.
Embolization. The doctor cuts off the blood supply to the area where the tumors are located, causing the cancer cells to die.
Destruction of tumor cells by radiofrequency ablation.
Chemotherapy and/or radiation therapy.

Proton pump inhibitors and H2 antagonists

Proton pump inhibitors (PPIs) and H2 antagonists (anti-H2) reduce acid production in the stomach, relieving symptoms caused by massive gastrin production.
These drugs are effective, but only if they are taken in high doses and for long periods.

Long-term treatment with PPIs (for example, drugs Esomeprazole, Omeprazole, Rabeprazole) and anti-H2 (for example, Ranitidine, Nizatidine) can cause serious side effects in people over 50 years of age, such as hip, wrist and/or vertebral fractures.

If the tumor has metastasized to the liver

If liver metastases have been concentrated in only one area of the liver and isolated from the rest of the organ, they can be surgically removed.
In the absence of such conditions, the only possible remedy is liver transplant, a delicate operation and not without possible complications.

Other treatments

If ulcers penetrate the stomach, a special intervention is planned.
If they cause severe blood loss, immediate blood transfusion for .

Forecast

If the diagnosis is made in a timely manner, there is a high probability of a positive surgical intervention (since the tumor probably appeared in a single form), which will cure the patient completely.

In cases where the gastrinoma is not removed on time, is in a hard-to-reach place, or is associated with MEN1, surgical intervention becomes impossible, so the patient is forced to live with the symptoms characteristic of Zollinger-Ellison syndrome (diarrhea, etc.).

Interesting

Higher education(Cardiology). Cardiologist, therapist, functional diagnostics doctor. Well versed in the diagnosis and treatment of diseases respiratory system, gastrointestinal tract and cardiovascular system. Graduated from the academy (full-time), has a lot of work experience behind her.

Specialty: Cardiologist, Therapist, Doctor of Functional Diagnostics.

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