Which are already going through hard times, there arises special syndrome, inherent only in such pathologies. It is characteristic of carcinoids - tumors consisting of neuroendocrine cells.

Due to the slow progression of the disease, some people may full recovery, but with early diagnosis and surgery.

Carcinoids and carcinoid syndrome

Carcinoids are so named because of their morphological similarity to carcinomas, but they differ from the latter in a lower degree of malignancy.

Currently, they account for up to 9% of gastrointestinal cancers, but can form in any part of the body (approximately 0.2% of the total).

Tumors form from cells nervous system and are hormonally dependent, since they themselves produce serotonin and some other biologically active proteins. The age of patients diagnosed with such diseases is usually at least 50-60 years.

Carcinoids grow slowly over many years. Their sizes are often small, so until recently they were considered almost . Often they were revealed only after an autopsy, and were not the cause of death.

But it has now been proven that in 80% of cases without treatment, these neoplasms spread into the intestines and organs. abdominal cavity, .

Carcinoid syndrome– a set of clinical manifestations that are entirely due to the presence of carcinoid in humans. Since these tumors secrete hormones, the latter enter the blood and contribute to the development of the entire pathological symptom complex.

This phenomenon does not occur in every patient, but it can last several years and include different symptoms from the skin, abdomen, heart, etc.

Photo of a patient with carcinoid syndrome

Carcinoid syndrome is more likely to develop in the presence of a tumor small intestine and bronchi, since it is these neoplasms that produce a significant amount of hormones.

Since other tumors of the gastrointestinal tract and other parts of the body produce less such substances, their symptoms may be absent until last stage. Only the presence of metastatic processes changes the picture, reflected in the appearance of the clinical picture.

Types of carcinoids

Up to 40% of tumors that cause a characteristic syndrome are located in the small intestine, up to 25% in the appendix, 15% near the anus. Less common are neoplasms of the described type in the stomach, bronchi, and reproductive system.

The main characteristics of carcinoids, depending on location, are as follows:

1. . It develops on the bottom or fornix of the organ; when gastric juice is released, it provokes the release of histamine, a mediator of allergy. In the end hydrochloric acid produced in increased quantity. Women get sick more often. The symptoms resemble those of an ulcer - pain, heartburn, and sometimes bleeding. There are three types of formations - those arising from atrophic gastritis, from Zollinger-Ellison syndrome, without connection with any diseases.
2. . They are often diagnosed by chance, as they rarely cause symptoms. At large sizes clinical hemorrhoids may be observed, as well as constipation and rectal bleeding. Even if metastases form, carcinoid syndrome is unlikely.
3. Bronchial tumor. They occur infrequently, up to 2% of neoplasms in the lower respiratory tract. Many patients - heavy smokers, and in the latter, usually slowly progressing tumors acquire an aggressive course, often spreading to the diaphragm area.
4. . The most common type of localization. Almost always on late stages gives a complex of characteristic symptoms, and also manifests itself as metastases and obstruction. Can lead to intestinal ischemia (oxygen starvation due to poor circulation), kinks and deformation. These tumors are very difficult to diagnose.
5. Tumor of the appendix (appendix). They are observed more often in young people, in the vast majority of cases they are discovered when the cecum is removed due to its inflammation. They are small in size, almost do not metastasize, and therefore are often considered benign.
6. . Represents up to 2% of total number cancerous lesions of the lung parenchyma. Smoking is often cited as the cause. Mostly, cancer is detected in people over 60 years of age and males. There are several types of such carcinoids - highly differentiated, poorly differentiated, atypical. The prognosis of the last two types is poor, the degree of malignancy is high.

Causes of the disease

The etiology of carcinoids has not yet been clarified. However, predisposing factors are considered:

• Heredity
• Bad ecology
• Working in hazardous industries
• Excessive consumption of fried foods and smoked foods
• Atrophic gastritis and other precancerous diseases

The immediate cause of the development of the entire symptom complex is the production of hormones, hormone-like substances and allergy mediators by the emerging tumor.

Since this complication does not occur in every patient, the following conclusion was made: the syndrome is typical only for metastatic tumors, or for large neoplasms that have crossed the liver barrier. This is important because all tumor products enter the systemic circulation and are not “extinguished” by the liver.

Typically, the following substances are found in the bloodstream of a sick person:

• Histamine
• Prostaglandins
• Serotonin
• Bradykinin
• Enteramine
• Chromogranine

An excess of such components, of which the most irritating is serotonin, leads to the development of symptoms of carcinoid syndrome. In particular, hormones can constrict blood vessels, which causes an increase in blood pressure.

The interaction of a pathologically large amount of released substances and other normal hormones in the body also causes heart damage with the development valvular insufficiency, and other serious violations.

Symptoms

Signs carcinoid syndrome are varied and will largely depend on what specific substances were produced by the neoplasm.

In the vast majority of cases it occurs severe redness skin, which is most noticeable on the front of the head, on the front of the neck, and on the décolleté. The body temperature of many patients also does not remain normal - it increases.

Not everyone has skin hyperemia all the time. Some suffer from sudden hot flashes in the upper body and head only after overload, nervous overstrain, drinking alcohol, spicy food. Paroxysmal redness of the skin and heat can go away spontaneously after a couple of minutes or after 2-5 hours.

If you measure the pulse at such a moment, it will be increased, and the pressure, on the contrary, will be reduced. Hypertension rarely occurs during attacks.

Another symptom that occurs in almost 90% of cases is frequent diarrhea. Diarrhea occurs without a combination with hot flashes, or develops along with them. If, at the time of the onset of diarrhea, you take drugs that inhibit serotonin, then this unpleasant symptom ends.

Sometimes, instead of diarrhea, intestinal obstruction develops with severe pain in the peritoneum, if the neoplasm is already so large that it blocks the intestinal lumen.

Others possible symptoms, united by carcinoid syndrome:

• Swelling, dropsy of the abdomen, enlarged liver, its pain, severe shortness of breath, including in a lying position - signs of heart failure.
• Bronchospasm, wheezing in the chest, breathing problems as a result of bronchial compression or the influence of tumor hormones.
• Dizziness and fainting as a result of oxygen starvation of brain tissue.

Over time, the intensity of symptoms increases, as does the frequency of acute manifestations. A complication can be a crisis state with severe hypotension up to shock, with suffocation due to bronchial spasm. The immediate cause of this consequence is surgery on the tumor.

Diagnosis of the syndrome

The main indicator that reflects the occurrence of carcinoid syndrome is an increase in serotonin in the blood.

In addition, the patient has very high levels of 5-hydroxyindoleacetic acid in his urine.

In order for the tests to be accurate and objective, a few days before they are performed, a person is prohibited from eating foods with a high presence of serotonin.

If all the symptoms are too obvious, but the analysis does not give positive result, a test with provocation (for example, drinking alcohol) is required.

After identifying markers of carcinoid syndrome, it is urgent to do a detailed examination and find the area where the tumor is localized.

The following methods may be needed to search:

• X-ray
• , MRI
• Endoscopic and standard ultrasound
• Capsule endoscopy of the intestine
• Fibrogastroduodenoscopy
• Fibervideocolonoscopy
• Bronchoscopy
• Laparoscopic examinations

Since the syndrome develops only in 15-20% of patients, it must be distinguished from manifestations of menopause, hypertension, side effects taking medications.

Treatment of the disease

The best option for effective treatment is to remove the tumor at an early stage or as it is detected. This is the only way to completely eliminate carcinoid syndrome.

But sometimes surgery is impossible, since tumor metastases already exist in the liver or in a number of other organs. However, attempts are always made to remove at least some of the new tumors, which will reduce the overall volume of abnormal hormone-producing tissue.

Use for this type of cancer does not make sense. renders weak therapeutic effect, but at complete absence the possibility of operating on it is still prescribed. A cytostatic is more often recommended, which works in half of the clinical cases.

Regarding symptomatic treatment syndrome, it is advisable to carry it out by taking drugs that inhibit serotonin (for example, Deseril).

Other means to reduce discomfort are:

• Antihypertensive drugs
• Opioid analgesics
• Astringents
• Antidiarrheal drugs
• Glucocorticosteroids
• Antidepressants
• Antihistamines
• Interferons

Patients should avoid eating foods rich in serotonin (bananas, nuts, etc.). This will help reduce intoxication with this hormone.

Forecast

The average survival time with the syndrome is 5-15 years due to the slow growth of tumors (50% of people live more than 5 years). If the operation is successfully performed, the prognosis is favorable.

Very rarely there is a fulminant course of the disease with fatal. Most poor prognosis with bronchial carcinoid.

Video about modern diagnosis and treatment of carcinoid syndrome:

This is a combination of symptoms caused by the presence of a neuroendocrine tumor (carcinoid) in the body and the release of large amounts of hormones into the blood. Carcinoids produce about forty biologically active substances, But highest value among them are serotonin, histamine, tachykinin, kalikrein and prostaglandin. Neuroendocrine tumors can occur in any organ of the digestive system (usually in the small intestine), as well as in the bronchi.

Signs of carcinoid syndrome

Sudden redness of the skin of the face, neck and chest caused by a rush of blood to these areas of the body is hallmark carcinoid syndrome. This symptom occurs in approximately 85% of people with neuroendocrine tumors.

The course of such an attack differs in patients with different tumor locations. Thus, in patients with intestinal carcinoid, such flushes of blood to the face and torso occur suddenly and last from thirty seconds to half an hour. At this time, a person is overtaken by a feeling of heat. Severe attacks accompanied by a fall blood pressure and increased heart rate. As the disease progresses, attacks may become more frequent and prolonged, and the skin may become bluish.

The syndrome can occur spontaneously, or be provoked by food intake, alcohol, stress, bowel movements, anesthesia and other factors. Attacks triggered by anesthesia can last for hours and be accompanied by a pronounced drop in blood pressure. This phenomenon in medicine is called carcinoid crisis.

Another sign of carcinoid syndrome is the appearance of venous telangiectasia on the skin. This is an extension small vessels, which appears in the form spider veins or meshes on the skin. Telangiectasia in carcinoid syndrome often occurs in the area of ​​the upper lip, nose, and cheekbones.

No less significant symptom is diarrhea, which is reported in approximately 80% of patients with neuroendocrine tumors. The frequency of stool can vary from several times to thirty per day. The stool is usually watery, without blood. Diarrhea may be accompanied by abdominal pain.

Approximately 10-20% of patients complain of shortness of breath and wheezing, most often occurring during attacks of redness of the skin of the face and torso.

Carcinoid syndrome is characterized by the deposition of fibrin plaques on the heart valves, chamber walls, and also on the internal walls pulmonary artery and aorta. The right side of the heart is most often affected. Such changes lead to valvular insufficiency. This symptom occurs in 40% of patients.

If the carcinoid tumor is localized in the stomach or bronchi, the course of an attack of carcinoid syndrome may vary. When the tumor is localized in the stomach, the skin may turn red in spots, and the person is bothered by severe itching. Diarrhea and heart damage are rare. Symptoms of carcinoid syndrome with this tumor localization are caused by the biologically active substance histamine.

In patients with a carcinoid tumor localized in the bronchi, hot flashes are more severe and prolonged, sometimes lasting several days. These attacks may be accompanied by disorientation, anxiety, and tremor (shaking of body parts). In addition, such patients experience symptoms such as:

1. Swelling of the skin around the eyes;
2. lacrimation;
3. Increased salivation;
4. Hypotension (low blood pressure);
5. Rapid heartbeat;
6. Diarrhea;
7. Dyspnea;
8. Oliguria (decreased urine volume).

It is believed that these symptoms in bronchial carcinoid tumors are caused by the influence of histamine secreted by the tumor itself.

Symptoms

There are also less significant symptoms that occur with carcinoid tumors. Thus, a violation of tryptophan metabolism with excessive formation of serotonin can lead to vitamin PP deficiency, which manifests itself in the form of pellagra. This disease is characterized by symptoms such as diarrhea, dermatitis (inflammation of the skin) and dementia (dementia).

Carcinoid tumors can also affect protein metabolism. As a result of protein synthesis disorders, muscle weakness and even atrophy.

Patients with severe bouts of redness may experience persistent swelling in the face and, less commonly, in the extremities.

Diagnostics

Symptoms of carcinoid syndrome are not specific, that is, they can also appear in other diseases, for example, IBS (irritable bowel syndrome) or colitis, so it is extremely important to determine that the change in the patient’s condition is caused precisely by the presence of tumors in the body.

To test for carcinoid syndrome caused by serotonin-secreting tumors, a test is performed to test for the presence of a tumor waste product, 5-hydroxyindoleacetic acid (5-HIAA), in the urine. To exclude a false positive result, the patient is prohibited three days before the test from taking foods that contain serotonin (tomatoes, pineapples, bananas, avocados, plums, walnuts, eggplants and other products) as well as medicines that contain it.

Treatment

For treatment, medications are prescribed that suppress the production of neuroendocrine tumor hormones or those that reduce their effects. Some symptoms, particularly hot flashes, can be relieved with somatostatin medications (octreotide). In addition, hot flashes can be combated with the help of phenothiazines (chlorpromazine), phentolamines.

Loperamide and diphenoxylate can be prescribed to treat diarrhea. To prevent pellagra, food intake is prescribed, rich in vitamin RR and also synthetic analogue vitamin

To prevent the conversion of 5-hydroxytryptophan to serotonin, patients are prescribed methyldopa or phenoxybenzamine.

Information about octreotide, loperamide and others medicines can be studied in electronic directory

Historical background: 1868 – Lanhans first described a carcinoid tumor; 1907 – Oberndofer first used the term “carcinoid”; 1930 - Cassidy described a disease accompanied by watery diarrhea, cardiac dysfunction, shortness of breath, redness of the facial skin; 1931 - Scholte first suggested a connection between a tumor of the small intestine and damage to the valve leaflets right half hearts; 1949 - Rapport proposed the structural formula of serotonin; 1952 - Bjork, Aksen, Thorson first described the clinical manifestations of carcinoid syndrome in a boy who suffered from pulmonary valve stenosis, tricuspid valve insufficiency and unusual cyanosis; 1954 - Waldenström and Pernov discovered hyperserotoninemia in patients with metastatic carcinoid.; 1960 – Harris observed a persistent hypertensive reaction when serotonin was administered to humans.

Carcinoid syndrome- this is a disease caused by the circulation of mediators, which is manifested by redness of the skin (90%), diarrhea (75%), noisy breathing (20%), endocardial fibrosis (33%), damage to the valves of the right heart; sometimes pleural, peritoneal or retroperitoneal fibrosis occurs.

The development of carcinoid syndrome is caused by a hormonally active tumor arising from enterochromaffin (argentaffin) cells of the intestine (Kulchitsky cells). In this case, increasing intestinal disorders are accompanied by hypertensive syndrome (Gogin E.E., 1991).

Until 1953, every case of carcinoid syndrome was considered casuistic. However, since the description of the clinical picture of the disease, the frequency of detection of carcinoid tumors has increased significantly. However, intravital diagnosis of carcinoid syndrome is quite rare. According to autopsy materials, carcinoids occur in 0.14–0.15% of cases.

Most often, carcinoid tumors are localized in the gastrointestinal tract, less often they occur in the bronchi, gall bladder, pancreas and ovaries. The sizes of tumors, as a rule, are not large and vary from 0.1 to 3 cm. The catcinoid cell is polygonal, has chromatin and large number randomly arranged granules. The connective tissue stroma of a carcinoid tumor contains a significant amount mast cells, which have the ability to concentrate and possibly produce serotonin.

The basis of pathogenesis Carcinoid syndrome is due to the production of 5-hydroxytryptamine (serotonin, enteramine) by tumor cells, which causes hyperserotoninemia and an excess of other physiologically active substances. The classic symptoms of carcinoid syndrome with a significant increase in blood pressure develop only when the primary focus or metastases of the tumor are outside the hepatic barrier. In these cases, the serotonin produced by tumor cells is not destroyed and enters the general circulation through the hepatic vein, causing various pathological conditions.

The vasoconstrictor effect of serotonin was the first of its properties to become known. Most researchers have found a hypertensive reaction when administering serotonin to humans. However, there is a sufficient number of studies indicating that after giving serotonin there is a decrease in blood pressure. The nature of the vascular reaction in response to serotonin is influenced by the method of its administration. Thus, when administered intravenously, an increase in minute volume and blood pressure is observed, and when serotonin is administered intraperitoneally, a hypotensive reaction is observed. It is believed that the variability in blood pressure responses to serotonin is associated with the ability of the vascular bed to respond differently to this amine.

It is known that there are close interactions between serotonin and other biological amines. For example, it is considered proven that the development vascular disorders in carcinoid syndrome is due to the interaction of serotonin and catecholamines (Menshikov V.V. et al., 1972). Cases of a combination of carcinoid syndrome with acromegaly and hypercortisolism have been described. It is possible that this combination is due to the ability of carcinoid tumor cells to produce GH- and ACTH-like substances.

Under the influence of high concentrations of serotonin Fibrosis of endothelial tissue develops, smooth muscle contraction occurs, which causes narrowing of the arteries. Damage to the cardiovascular system due to hyperserotoninemia in carcinoid syndrome is reduced to the development of tricuspid valve insufficiency and pulmonary artery stenosis, as well as fibrosis of the endocardium of the right atrium and right ventricle. Narrowing of arterioles leads to the development of arterial hypertension.

Carcinoid tumors are classified as arising from anterior intestinal tube(bronchi, stomach, duodenum, bile ducts, pancreas), middle part of the intestinal tube(jejunum and ileum, appendix, ascending colon), posterior part of the intestinal tube (transverse colon, descending, sigmoid and rectum). Sometimes they are also found in the gonads, prostate, kidneys, mammary glands, thymus gland or skin.

For clinical picture Carcinoid syndrome is characterized by the presence of vasomotor disturbances of the “hot flash” type. In this case, certain areas of the skin become red, sometimes cyanotic, and the patient feels heat in these places. In addition to violations vascular tone and heart damage is marked abdominal syndrome, which is manifested by the presence of diarrhea and pain along the intestines. The effect of serotonin on the smooth muscles of the bronchi can lead to a sharp narrowing of the latter and the development of asthmatic attacks. As a rule, the course of the disease is paroxysmal. The duration of attacks varies significantly - from 30 seconds to 10 minutes, and in some cases attacks can be repeated several times during the day. Their occurrence is most often provoked in connection with nervous or physical stress, or alcohol intake. A number of symptoms, such as cyanosis, telangiectasia, oliguria and cardiac damage, persist for a long time and determine the condition of patients in the interictal period.

The most characteristic changes in the blood picture in carcinoid syndrome, there is an acceleration of ESR and leukocytosis, and a direct relationship is determined between changes in the blood and the occurrence of crises. Carcinoids, especially bronchial carcinoids, can secrete ACTH, which causes Cushing's syndrome, growth hormone-releasing hormone, which causes acromegaly.

The diagnosis is confirmed high levels of sorotonin in the blood plasma, increased urinary excretion of 5-hydroxyindoleacetic acid, as well as detection of intestinal, liver, and lung tumors during instrumental methods research. Biochemical indicators can be distorted when consuming foods containing large amounts of serotonin (bananas, walnuts, citrus fruits, kiwi, pineapples), taking rauwolfia preparations, phenothiazines, salicylates, as well as in pathological conditions complicated intestinal obstruction, when the excretion of 5-hydroxyindoleacetic acid can increase to 9-25 mg/day.

Immunofluorescence studies confirm the presence of neuropeptides (serotonin, substance P and neurotensin) in carcinoid tumors. Definition carcinoembryonic antigen(CEA) also has a certain meaning in the diagnosis of carcinoid tumors: its level is usually normal or minimal. If the CEA concentration is elevated, another tumor should be suspected.

Comprehensive topical diagnostics carcinoids involves X-ray examination, computed tomography, ultrasound scanning, the use of immunoautoradiographic methods for determining hormones in the blood, selective arterio- and venography, scintigraphy with indium-111-octreotide and morphological examination of the biopsy specimen. The use of the entire complex of modern research methods (fluoroscopy, endoscopy, selective arterio- and phlebography, ultrasound, computed tomography, radionuclide studies, determination of hormones in the blood, immunohistochemical study of tumor biopsies) ensures the correct diagnosis in 76.9% of cases.

Considering general principles therapy carcinoid syndrome, it should be noted that it is advisable to most early removal tumors. However, it must be remembered that clinical manifestations of carcinoid syndrome very often occur in cases where there are already functionally active metastases in the liver. In this case, radical surgical treatment not feasible. However, mitigation of the clinical picture of carcinoid syndrome can be achieved by excision of as much of the metastases as possible, thus reducing the total amount of serotonin-producing tissue. If surgery is not possible for any reason, radiotherapy can be used, the effectiveness of which, however, is low due to the resistance of carcinoid tumors to radiation exposure. After radiation therapy, no reliable data on an increase in life expectancy have been obtained.

Among medications used in the treatment of carcinoid tumors, cyclophosphamide should be highlighted, the effectiveness of which, according to a number of authors, is about 50%. There are also reports that a good therapeutic effect is observed when prescribing serotonin antagonists, of which the most wide application found cyproheptadine and deseryl. Cyproheptadine has not only antiserotonin, but also antihistamine effect. It is prescribed intravenously in a dose of 6 to 40 mg. Deseril is used orally at a dose of 6 to 24 mg and intravenously for 1-2 hours at a dose of 10-20 mg.

Some antidepressants- fluoxetine, sertraline, fluvoxamine, paroxetine can be used for symptomatic therapy.

Histamine H1 and H2 receptor blockers(cimetidine, ranitidine, diphenhydramine/diphenhydramine) are effective for carcinoids that produce predominantly histamine. Loperamide (Imodium) is used to relieve diarrhea.

Synthetic analogues of somatostatin Octreotide (Sandostatin) and lanreotide (Somatulin) are widely used to treat carcinoid syndrome. Sandostatin, interacting with somatostatin receptors, suppresses the production of active substances by the tumor. The recommended regimen for the use of sandostatin (octreotide) in the treatment of carcinoid and neuroendocrine syndromes is 150-500 mcg subcutaneously 3 times a day. The drug increases the survival rate of patients with carcinoid tumors and the presence of metastases, improves the quality of life of patients, relieving or eliminating hot flashes, intestinal motor dysfunction, and diarrhea.

Numerous studies have been conducted on the use of interferon α in patients with carcinoid tumors. To treat patients, doses of α-interferon 3-9 IU were used subcutaneously 3-7 times a week. The use of higher doses of the drug does not improve treatment rates, but significantly increases the percentage of toxic reactions. When treated with α-interferon, improvement is observed in 30-75% of cases.

Treatment of arterial hypertension carried out according to general rules. In some cases, treatment should be aimed at combating complications of carcinoid syndrome, such as cardiovascular failure, gastrointestinal bleeding etc. It should be noted that the duration of the disease (10 years or more), significant exhaustion of patients with carcinoid syndrome requires the appointment of restorative therapy.

Carcinoid syndrome is a symptom complex resulting from the release of tumors various hormones and getting them into the bloodstream. After such an introduction, many readers will decide that they are waiting for a description of another cancer: symptoms, signs, treatment methods, and in the end - a prognosis for the development of events (of course, unfavorable) and a non-binding phrase regarding the need to gather one's will and believe in a miracle. After all, even those of us who are very far from medicine “know” perfectly well that a tumor is incurable, and cancer is an inexorable sentence that cannot be appealed.

Dear readers! If you want to find confirmation of your fears, then we have to disappoint you: you have come to the wrong address. And the point here is not at all that we strive to give you only positive, encouraging information. Quite the contrary: there are plenty of resources on the Internet that either claim that cancer (we especially note, any cancer!) can be perfectly cured, or gradually prepare the patient for the transition to another world, offering to come to terms with the inevitable and think about something eternal.

We prefer a different approach: to provide the most accurate information regarding a particular disease. Yes, it's a tumor special case: menacing symptoms, obvious signs of non-binding duty sympathy in the eyes of the doctor and the horror that envelops the patient after the next visit to the oncology clinic. An exhaustive analysis of such misconceptions requires a separate discussion, especially since the carcinoid syndrome that we will talk about today is not, in fact, a malignant neoplasm. This, we repeat, is a complex of symptoms explained by the release of hormones into the bloodstream.

The essence of the problem

Carcinoid tumor - a special type malignant neoplasms, developing from cells of the endocrine and nervous systems. It differs from other types of cancer in that it releases large amounts of certain hormones (serotonin, histamine, bradykinin and prostaglandins) into the bloodstream. Because of this, the patient, in addition to the “usual” oncological signs, some specific symptoms are also observed that are in no way related to the location of the tumor: hyperemia, diarrhea and stomach cramps, which are called “carcinoid syndrome”.

But in order to once and for all close the topic of the “incurability” of such tumors, it should be especially clarified: carcinoids grow and spread very slowly, due to which metastases (the main cause of mortality in oncology) appear much later than with ordinary types of cancer. Which leads us to a very obvious conclusion: when early diagnosis and with timely treatment, a fatal outcome can often be avoided. But for this you will have to undergo regular preventive examinations, refuse bad habits and start taking care of your health. Agree, it’s much “easier” to pretend for 5-7 years that nothing is happening, and when the doctor informs you that you have a few months left to live, start mourning your own bitter fate. Naturally, the oncologist will be “to blame” for such a development of events, and not the patient himself...

Possible locations

1.Tumor of the small intestine (10% of cases). It is quite rare, which is “compensated” by the great difficulties in detecting it. Long time it may not manifest itself in any way (in exceptional cases, the problem is revealed by X-ray examination). The diagnosis is usually made after the appearance of metastases, involvement in pathological process liver and, as a result, a significant worsening of the prognosis. Greatest threat for life it represents obstruction of the small intestine, and in some cases - the death of part of it (necrosis) or rupture. Characteristic clinical manifestations are nausea, diarrhea, vomiting, paroxysmal pain in the abdominal area and decreased patency of blood vessels.

2. Appendicular tumor. It is found in less than 1% of cases when the appendix is ​​removed. Such neoplasms rarely reach a significant size (usually no more than 1 cm in diameter) and most often develop without any clinical manifestations. If an appendiceal tumor is detected during surgery to remove the appendix, the likelihood of recurrence is extremely low and the patient's prognosis is good. Large neoplasms (at least 2 cm) turn out to be malignant in approximately 30% of cases, but they are relatively uncommon.

3. Rectal tumor. Carcinoid syndrome in this case develops very rarely, and the likelihood of metastases directly depends on the size of the tumor. If the tumor diameter is less than 1 cm, this happens in 1-2% of cases, but with larger sizes (2 cm or more) the risk increases to 60-80%.

4. Gastric (gastric) tumor. There may be three types, which differ in size, nature of the tumor and the likelihood of developing metastases.

• Type I (size less than 1 cm). Almost always, such a neoplasm is benign, due to which the likelihood of metastases tends to zero. Most often found in patients with chronic atrophic gastritis or pernicious anemia. With timely diagnosis and timely surgery, the prognosis is favorable.
• Type II (from 1 to 2 cm). It is very rare, and the chance of transformation into a malignant form is extremely low. The risk group is patients with MEN (multiple endocrine neoplasia, a rare genetic disorder), and in this case similar tumors also form in the pancreas, parathyroid glands or epiphysis.
• Type III (more than 3 cm). Almost always, such tumors are malignant, and the risk of spreading into surrounding tissues and organs is extremely high. The prognosis is most often unfavorable, and the later the correct diagnosis is made, the worse the prospects.

5. Colon tumor. The usual location is right side end part digestive tract. Most often it is detected too late, when the transverse size of the tumor is more than 5 cm, and metastases are observed in two thirds of patients. The prognosis is unfavorable.

Symptoms

Speaking about clinical manifestations, it should be understood that they can be of two types. Carcinoid syndrome has some symptoms, but its root cause, a tumor, has others. It often happens that during the initial examination, the clinical manifestations are “mixed”, and the doctor begins treatment for flushing, diarrhea, pain in the heart and abdomen, carcinoid crisis and wheezing, without knowing what causes them. At the same time, the oncological nature of the disease may remain in the shadows, and the patient will learn about his true diagnosis much later. Therefore, in this case, an individual approach to each patient is extremely important, and the symptoms themselves (will be discussed below) should be considered in their entirety.

1. Hyperemia. Occurs in 90% of patients and is an obvious (but by no means unique) sign of the syndrome. It is assumed that bradykinin and serotonin are “responsible” for hyperemia, although this issue has not yet been fully clarified. Characteristic clinical manifestations:

• elevated temperature;
• redness of the skin of the face and neck;
• rapid heartbeat in the background reduced performance blood pressure;
• attacks of dizziness.

2. Diarrhea. It is observed in 75% of patients diagnosed with carcinoid syndrome. Often (but not always) occurs against the background of hyperemia. The main “culprit” is serotonin, so specific medications that suppress the action of this hormone (ondansetron, zofran) can alleviate the patient’s condition. IN in rare cases diarrhea occurs due to a nearby tumor or due to the influence of a metastatic focus.

3. Pathologies cardiovascular system. Occurs in every second patient. Most often, the pulmonary valve suffers, impaired mobility of which significantly reduces the ability of the heart to pump blood from the right ventricle to other organs and systems of the body. Possible clinical manifestations (explained by excessive production of serotonin):

• liver enlargement;
• swelling of the extremities (most often the legs are affected);
• accumulation of fluid in the abdominal cavity (ascites).

4. Carcinoid crisis. An acute and life-threatening condition that may occur during surgery. Therefore, if a patient with confirmed carcinoid syndrome requires surgery, pre-treatment with somatostatin is mandatory. Main symptoms of carcinoid crisis:

• sudden and sharp drop in blood pressure;
• life-threatening increase in heart rate;
• increased blood glucose levels;
• severe bronchospasm.

5. Wheezing (10% of patients). Are a direct consequence of spasm respiratory tract, but upon a superficial examination can be explained by problems with the respiratory system.

6. Severe pain in the stomach. It is explained either by liver metastases or sudden intestinal obstruction. In the second case, the patient requires urgent surgery, and the doctor deals with the root cause that caused the obstruction (carcinoid syndrome and primary tumor) after eliminating the immediate danger to life.

7. Tides

The so-called tides deserve special mention. This is a specific symptom, manifested by a sudden feeling of heat, unmotivated anxiety, increased sweating and redness of the skin. Most older women are familiar with hot flashes, which often appear with the onset of menopause, but when describing carcinoid syndrome, the corresponding clinical signs look a little different:

• Type I (erythematous): the attack lasts 1-2 minutes and is limited to the face and neck;
• Type II: the face becomes cyanotic for 5-10 minutes, and the nose becomes red-purple;
• Type III: an attack can last several hours or days and is expressed in the appearance of deep wrinkles on the forehead, dilation of conjunctival vessels, severe lacrimation, diarrhea and attacks of hypotension;
• Type IV: spots appear irregular shape bright red, usually on the arms and around the neck.

Approximate correspondence between the types of hot flashes and the area of ​​tumor localization:

• Types I and II: middle part of the digestive tube, bronchi, pancreas;
• Type III: anterior part of the digestive tube;
• Type IV: stomach.

Diagnostics

1. Biochemical studies show:

• high levels of serotonin in blood plasma;
• increased secretion of 5-hydroxyindosculic acid in the urine.

But here it is important to understand that the final results can sometimes be seriously distorted. The main factors influencing their reliability are the following:

• consumption of certain foods (walnuts, kiwi, bananas, citrus fruits, pineapples);
• taking certain pharmacological drugs;
• intestinal obstruction.

2. Immunofluorescence study will reveal:

• the presence of neuropeptides (substance P, serotonin, neurotensin);
• change in the level of CEA (carcinoembryonic antigen).

3. Instrumental studies

• scintigraphy ( intravenous administration radioactive isotopes of indium-111 followed by visualization on a gamma tomograph);
• endoscopic examination internal organs through thin probe with a miniature camera at the end (this procedure is often combined with a biopsy);
• selective arterio- and phlebography;
• X-ray examination;
• CT and MRI to confirm the diagnosis.

Unfortunately, carcinoid syndrome is one of those pathologies for which 100% has not yet been developed. effective method diagnostics Because even comprehensive examination guarantees production correct diagnosis only in 70% of cases. But what about characteristic symptoms and clinical manifestations, you ask? Unfortunately, they cannot be called at all unique. They may be useful in developing treatment tactics, but will be of little help in making a diagnosis and confirming it.

Treatment

A patient diagnosed with carcinoid syndrome may be offered various options therapy:

1. Radical surgery. The main treatment method with proven effectiveness and a high survival rate. The volume and type of intervention is determined both by the localization of the primary tumor and the presence or absence of metastases:

• skinny and ileum: resection of the affected area, the corresponding area of ​​the mesentery and, possibly, nearby lymph nodes(survival rate: 40 to 100%);
• ileocecal sphincter: right hemicolectomy;
• mesenteric lymph nodes (large carcinoids from 2 cm), colon: hemicolectomy;
• appendix: appendectomy.

2.Palliative surgery. During this procedure, the primary tumor node and the largest metastases are removed. There is no need to talk about a cure in this case, but it is possible to improve the quality of life.

3. Minimally invasive intervention: embolization or doping of the hepatic artery. The procedure gives a high (60 to 100%) chance of getting rid of hot flashes and diarrhea.

4. Chemotherapy. It is considered desirable when generalizing the process, in combination therapeutic measures after surgery and subject to the presence of certain unfavorable factors(heart damage, high excretion of 5-HIAA, liver dysfunction).

• the most effective drugs: streptozocin, doxorubicin, 5-fluorouracil, etoposide, dactinomycin, dacarbazine, cisplatin;
• the duration of the remission period is from 4 to 7 months;
• average effectiveness with monotherapy is less than 30%, in combination mode - up to 40%;
• for some types of tumors (anaplastic neuroendocrine), the effectiveness of chemotherapy increases significantly - up to 65-70%.

5. Radiation therapy. Efficacy in the treatment of carcinoid syndrome has not yet been proven, although integrated approach, aimed at maximizing life extension, should not be abandoned.

6. Symptomatic treatment

• serotonin antagonists: methysergide, cyproheptadine;
• selective serotonin reuptake inhibitors: citalopram, dapoxetine, fluoxetine;
• antidepressants: fluoxetine, fluvoxamine, sertraline, paroxetine;
• H1 and H2 receptor blockers: cimetidine, diphenhydramine, ranitidine;
• synthetic analogues of somatostatin: octreotide, sandostatin, lanreotide;
• alpha interferon (improvement in 30-75% of cases).

Risk factors

Forecast

Given the extremely slow growth of the primary tumor, effective surgical intervention and some improvement in prognosis with complex chemotherapy, most patients can expect 10-15 years of full life. Detection of carcinoid syndrome on early stages often provides complete cure Therefore, you should not neglect preventive examinations. Believe me, it’s better to spend a few hours a year than to regret missed opportunities later.

Carcinoid syndrome is a complex of symptoms that occurs due to the release of hormones by carcinoid tumors (the most common neoplasms of neuroendocrine cells) and their entry into the bloodstream. Carcinoid tumors are slowly progressing neoplasms that are relatively rare. Carcinoid syndrome occurs in 15-20% of patients with carcinoids. All age categories of the population are susceptible to the development of the pathological process.

A characteristic sign of carcinoid syndrome is sudden flushing of the skin of the face, neck and chest.

Causes and risk factors

Carcinoid syndrome usually occurs in association with endocrinologically active neoplasms in the ileum, rectum, appendix, or other sites gastrointestinal tract, as well as in the pancreas, bronchi, etc.

Forms of the disease

Depending on the clinical manifestations, there are following forms pathologies:

• latent carcinoid syndrome (in the absence of clinical manifestations, but changes biochemical parameters blood and urine);
• carcinoid syndrome without identifying the primary tumor site and its metastases;
• carcinoid syndrome with identification of the primary focus of the tumor and its metastases or only metastases to the liver.

Depending on the location, carcinoid syndrome is distinguished with a tumor of the anterior, middle or posterior part of the digestive tube.

Symptoms

Symptoms depend on the hormones secreted by the tumor. Clinical picture carcinoid syndrome in intestinal tumors, as a rule, manifests itself only when they metastasize to the liver.

A characteristic sign of the pathology is sudden hyperemia of the skin of the face, neck and chest, caused by a rush of blood. Often, skin hyperemia occurs due to eating, drinking alcohol, or distress. The nature of hyperemia differs depending on the location of the carcinoid tumor. In patients with intestinal neoplasms, hyperemia may be accompanied by a feeling of heat, and as the disease progresses, the skin may acquire a cyanotic hue during attacks.

When the tumor is localized in the stomach, hyperemic spots may appear on the skin, accompanied by severe itching. If the carcinoid tumor is localized in the bronchi, the attacks are more severe and prolonged (up to several days) and may be accompanied by tremor and disorientation in space.

In addition to hyperemia, the appearance of spider veins or vascular networks on the skin (mainly in the nasolabial triangle, on the cheekbones).

One more typical manifestation carcinoid syndrome is diarrhea. The frequency of bowel movements can vary from several to several dozen times a day. Diarrhea is often accompanied by abdominal pain and gastrointestinal cramps.

In patients with developed valvular heart lesions, heart murmurs occur.

In addition, with carcinoid syndrome, the following may be observed:

• wheezing;
• dyspnea;
• lacrimation;
• swelling around the eyes;
• increased salivation;
• decreased libido;
• hypotension;
• tachycardia;
• oliguria, etc.

Diagnostics

To make a diagnosis of carcinoid syndrome:

• taking anamnesis;
• determination of excretion of 5-hydroxyindoleacetic acid (a metabolic product of carcinoid neoplasm) in daily urine;
• biochemical examination of blood and urine;
• Ultrasound of internal organs;
• computed or magnetic resonance imaging;
• positron emission tomography;
• venography, selective arteriography;
• endoscopic examination;
• biopsy with morphological, histological and immunohistochemical examination of the biopsy sample.

To avoid false positive results at laboratory analysis urine, three days before the test, foods that contain serotonin, as well as medications that can distort the test results, are excluded from the patient’s diet.

Carcinoid syndrome occurs in 15-20% of patients with carcinoids.

Electrocardiography and echocardiography are performed to detect cardiac damage.

If the diagnosis is in doubt, in order to provoke specific attacks (“hot flashes”), tests are carried out with pentagastrin, catecholamines, alcohol, etc.

It is necessary to carry out differential diagnosis with idiopathic anaphylaxis, side effects from taking medications, symptoms of menopause, irritable bowel syndrome, etc.

Treatment

Radical surgical treatment of a carcinoid tumor and its metastases eliminates the clinical manifestations of carcinoid syndrome. In case of unresectable neoplasm, the impossibility of surgical intervention due to general condition patient or for other reasons, as well as to eliminate signs of carcinoid syndrome with an undetected tumor, conservative treatment is indicated.

Symptomatic treatment of manifestations of carcinoid syndrome is carried out using drugs that inhibit the production of hormones by a carcinoid tumor, as well as glucocorticoids, antidepressants, etc.

Possible complications and consequences

Carcinoid syndrome in some cases is complicated by carcinoid crisis, which can occur against the background of:

• major surgery;
• stress;
• instrumental diagnostic examination;
• chemotherapy;
• anesthesia during surgery.

Signs of carcinoid crisis:

• sudden change in blood pressure (hypertension or hypotension);
• deterioration of general condition;
• frequent noisy breathing;
• tachycardia, thready pulse;
• disorders of the central nervous system, etc.

Carcinoid crisis occurs acutely and in the absence of an urgent set of measures intensive care may go into shock and coma.

Forecast

The prognosis for carcinoid syndrome depends on the prevalence oncological process. In the presence of only a primary tumor and its successful treatment, the average five-year survival rate is 95%; in patients with multiple distant metastases, this figure does not exceed 20%. If regional lymph nodes are involved in the pathological process, the five-year survival rate is reduced to 60-70%.

Upon successful completion surgical intervention the prognosis is favorable.

The prognostic factor for carcinoid syndrome is proliferative activity cells of the primary neoplasm. Carcinoid tumors with a low proliferation index have a more favorable prognosis.

Prevention

Specific measures to prevent carcinoid syndrome have not been developed. Nonspecific preventive measures include:

• healthy lifestyle;
• sufficient physical activity;
• giving up bad habits;
• balanced balanced diet;
• avoiding the influence of oncogenic substances on the body (including occupational hazards);
• timely diagnosis and treatment of internal diseases, etc.

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