Carcinoma syndrome symptoms. Clinical picture of neuroendocrine tumors. Carcinoid syndrome and other syndromes. Appendiceal carcinoid tumor

• Which doctors should you contact if you have Carcinoid Syndrome?

What is Carcinoid Syndrome

Carcinoids- the most common tumors are from neuroendocrine cells (cells of the APUD system). These cells are derivatives of the neural crest. They are widely distributed in the body and contain and secrete some biologically active peptides. Most often, carcinoids develop in the gastrointestinal tract (85%), especially in the small intestine, as well as in the lungs (10%), less often affecting other organs (for example, the ovaries). Intestinal carcinoids rarely (in 10% of cases) manifest clinically. This is explained by the rapid destruction of biologically active peptides in the liver. Only with metastases of intestinal carcinoid to the liver do symptoms appear in 40-45% of patients. As for ovarian and bronchial carcinoids, they are capable of causing a characteristic syndrome in the early stages of the disease.

Carcinoid syndrome- a combination of symptoms that arise due to the release of hormones by tumors and their entry into the bloodstream.

Pathogenesis (what happens?) during Carcinoid syndrome

Carcinoid tumors can appear where there are enterochromaffin cells, basically throughout the body. A larger proportion of carcinoid tumors (65%) develop in the gastrointestinal tract. In most cases, carcinoid tumors develop in the small intestine, appendix, and rectum. Carcinoid tumors develop most rarely in the stomach and colon; The pancreas, gallbladder, and liver are least likely to develop carcinoid tumors (even though carcinoid tumors usually metastasize to the liver).

Approximately 25% of carcinoid tumors affect the airways and lungs. The remaining 10% can be found anywhere. In some cases, doctors cannot locate the carcinoid tumor despite symptoms of carcinoid syndrome.

Carcinoid tumor of the small intestine

In general, tumors of the small intestine (benign or malignant) are rare, much less common than tumors of the colon or stomach. Small carcinoid tumors of the small intestine may not cause any symptoms other than mild abdominal pain. For this reason, it is difficult to determine the presence of a small bowel carcinoid tumor at an early stage, at least until the patient undergoes surgery. It is possible to detect only a small proportion of small intestinal carcinoid tumors in the early stages, and even then this occurs unexpectedly on x-ray. Typically, carcinoid tumors of the small intestine are diagnosed in late stages, when the symptoms of the disease have become apparent and usually after metastases have appeared.

Approximately 10% of carcinoid tumors of the small intestine cause carcinoid syndrome. Typically, the development of carcinoid syndrome means that the tumor is malignant and has reached the liver.

Carcinoid tumors often obstruct the small intestine when they become large. Symptoms of small intestinal obstruction include paroxysmal abdominal pain, nausea and vomiting, and sometimes diarrhea. Obstruction can be caused by two different mechanisms. The first mechanism is an enlargement of the tumor inside the small intestine. The second mechanism is twisting of the small intestine due to fibrosing mesenteritis, a condition caused by a tumor in which extensive scarring occurs in the tissues located in close proximity to the small intestine. Fibrosing mesenteritis sometimes obstructs the arteries that carry blood to the intestines, which can result in the death of part of the intestine (necrosis). In this case, the intestines can rupture, which is a serious threat to life.

Appendiceal carcinoid tumor

Although tumors in the appendix are quite rare, carcinoid tumors are the most common tumors in the appendix (about half of all appendiceal tumors). In fact, carcinoid tumors are found in 0.3% of removed appendixes, but most of them do not grow larger than 1 cm and do not cause any symptoms. In most cases, they are found in appendices that have been removed for reasons other than tumors. Many institutions believe that appendectomy is the most appropriate treatment for these small appendiceal carcinoid tumors. The chances that the tumor will recur after appendectomy are very low. Appendiceal carcinoid tumors larger than 2 cm in 30% can be malignant and form local metastases. Therefore, larger carcinoid tumors should be removed. A simple appendectomy will not help in this case. Fortunately, large carcinoid tumors are quite rare. Carcinoid tumors in the appendix, even with metastases to local tissues, usually do not cause carcinoid syndrome.

Rectal carcinoid tumors

Rectal carcinoid tumors are often diagnosed incidentally during plastic sigmoidoscopy or colonoscopy. Carcinoid syndrome is rare in rectal carcinoid tumors. The likelihood of metastases is related to tumor size; 60-80% chance of metastases for tumors larger than 2 cm. For carcinoid tumors smaller than 1 cm, 2% chance of metastases. In summary, small rectal carcinoid tumors are usually successfully removed, but larger tumors (greater than 2 cm) require extensive surgery, which can even lead to partial removal of the rectum in some cases.

Gastric (stomach) carcinoid tumors

There are 3 types of gastric (stomach) carcinoid tumors: type I, type II and type III.

Type 1 gastric carcinoid tumors are usually less than 1 cm in size and are benign. There are complex tumors that spread throughout the stomach. They usually appear in patients with pernicious anemia or chronic atrophic gastritis (a condition in which the stomach stops producing acid). The lack of acid causes the cells in the stomach, which produce the hormone gastrin, to release large amounts of gastrin, which enters the blood. (Gastrin is a hormone secreted by the body to enhance the activity of stomach acid. Acid in the stomach blocks the production of gastrin. In pernicious anemia or chronic atrophic gastritis, the lack of acid is the result of an increase in the amount of gastrin). In addition, gastrin also influences the transformation of enterochromaffin cells in the stomach into a malignant carcinoid tumor. Treatment for type 1 carcinoid tumors includes treatments such as somatostatin-containing drugs that stop gastrin production or surgical removal of the part of the stomach that produces gastrin.

The second type of gastric carcinoid tumor is less common. Such tumors grow very slowly and the likelihood of them becoming malignant is very low. They appear in patients with a rare genetic disorder called MEN (multiple endocrine neoplasia) type I. In these patients, tumors arise in other endocrine glands, such as the pineal gland, parathyroid gland and pancreas.

The third type of gastric carcinoid tumor are tumors larger than 3 cm that are isolated (appearing one or more at a time) in a healthy stomach. Tumors of the third type are usually malignant and there is a high probability of their deep penetration into the walls of the stomach and the formation of metastases. Type 3 tumors can cause abdominal pain and bleeding, as well as symptoms due to carcinoid syndrome. Type 3 gastric carcinoid tumors usually require surgery to remove the stomach and nearby lymph nodes.

Carcinoid tumors of the colon

Colon carcinoid tumors usually form on the right side of the colon. Like carcinoid tumors of the small intestine, carcinoid tumors of the colon are often discovered at advanced stages. Thus, the average tumor size at diagnosis is 5 cm, and metastases are present in 2/3 of patients. Carcinoid syndrome is rare in carcinoid tumors of the colon.

Symptoms of Carcinoid Syndrome

Symptoms of carcinoid syndrome vary depending on what hormones the tumors secrete. Typically these are hormones such as serotonin, bradykinin (promotes pain), histamine and chromogranin A.

Typical manifestations of carcinoid syndrome:

• Hyperemia (redness)
• Diarrhea
• Abdominal pain
• Wheezing due to bronchospasm (narrowing of the airways)
• Heart valve damage
• Surgery can cause a complication known as carcinoid crisis.

Hyperemia

Flushing is the most common symptom of carcinoid syndrome. In 90% of patients, hyperemia occurs during the disease. Flushing is characterized by redness or discoloration of the face and neck (or upper body), as well as an increase in temperature. Attacks of hyperemia usually occur suddenly, spontaneously, they can be caused by emotional, physical stress or alcohol consumption. Attacks of hyperemia can last from a minute to several hours. Congestion may be accompanied by a rapid heart rate, low blood pressure, or dizziness if blood pressure drops too much and blood is not getting to the brain. Rarely, hyperemia is accompanied by high blood pressure. The hormones that are responsible for hyperemia are not fully recognized; These may include serotonin, bradykinin and substance P.

Diarrhea

Diarrhea is the second important symptom of carcinoid syndrome. About 75% of patients with carcinoid syndrome experience diarrhea. Diarrhea often occurs along with flushing, but can also occur without it. In carcinoid syndrome, diarrhea is most often caused by serotonin. Medicines that block the action of serotonin, such as ondansetron (Zofran), often relieve diarrhea. Sometimes diarrhea in carcinoid syndrome can occur due to local exposure to a tumor that obstructs the passage of the small intestine.

Heart diseases

Heart disease occurs in 50% of patients with carcinoid syndrome. Carcinoid syndrome usually leads to fibrotic changes in the pulmonary valve of the heart. Impaired valve movement reduces the heart's ability to pump blood from the right ventricle to the lungs and other parts of the body, which can lead to heart failure. Typical symptoms of heart failure include: enlarged liver, swelling of the legs, and fluid accumulation in the abdomen (ascites). The cause of damage to the tricuspid and pulmonary valves of the heart in carcinoid syndrome, in most cases, is intense long-term exposure to serotonin.

Carcinoid crisis

Carcinoid crisis is a dangerous condition that can occur during surgery. A crisis is characterized by a sudden drop in blood pressure, which causes shock. This condition may be accompanied by an excessively rapid heartbeat, elevated blood glucose levels, and severe bronchospasm. Carcinoid crisis can be fatal. The best way to prevent a carcinoid attack is surgery using somatostatin before surgery.

Wheezing

Wheezing occurs in approximately 10% of patients with carcinoid syndrome. Wheezing is a consequence of bronchospasm (spasm of the airways), which occurs as a result of the release of hormones by a carcinoid tumor.

Abdominal pain

Abdominal pain is a common symptom in patients with carcinoid syndrome. Pain may occur due to metastases in the liver, due to the tumor affecting neighboring tissues and organs, or due to intestinal obstruction (read about small intestinal carcinoid tumor below).

Diagnosis of Carcinoid Syndrome

Diagnosis of carcinoid syndrome confirmed by an increase in the daily urine level of the serotonin metabolite - 5-hydroxyindoleacetic acid.

Treatment of Carcinoid Syndrome

Treatment of carcinoid syndrome: radical surgical removal of the tumor. For metastases that cannot be removed, therapy is prescribed with octreotide, a long-acting analogue of somatostatin.

For frequent attacks, methyldopa preparations are used (dopegit 0.25-0.5 g 3-4 times a day), opium preparations for diarrhea. It is possible to use prednisolone in a dose of up to 20-30 mg per day.

Carcinoids- the most common tumors are from neuroendocrine cells (cells of the APUD system). These cells are derivatives of the neural crest. They are widely distributed in the body and contain and secrete some biologically active peptides. Most often, carcinoids develop in the gastrointestinal tract (85%), especially in the small intestine, as well as in the lungs (10%), less often affecting other organs (for example, the ovaries). Intestinal carcinoids rarely (in 10% of cases) manifest clinically. This is explained by the rapid destruction of biologically active peptides in the liver. Only with metastases of intestinal carcinoid to the liver do symptoms appear in 40-45% of patients. As for ovarian and bronchial carcinoids, they are capable of causing a characteristic syndrome in the early stages of the disease.

Carcinoid syndrome- a combination of symptoms that arise due to the release of hormones by tumors and their entry into the bloodstream.

Pathogenesis (what happens?) during Carcinoid syndrome:

Carcinoid tumors can appear where there are enterochromaffin cells, basically throughout the body. A larger proportion of carcinoid tumors (65%) develop in the gastrointestinal tract. In most cases, carcinoid tumors develop in the small intestine, appendix, and rectum. Carcinoid tumors develop most rarely in the stomach and colon; The pancreas, gallbladder, and liver are least likely to develop carcinoid tumors (even though carcinoid tumors usually metastasize to the liver).

Approximately 25% of carcinoid tumors affect the airways and lungs. The remaining 10% can be found anywhere. In some cases, doctors cannot locate the carcinoid tumor despite symptoms of carcinoid syndrome.

Carcinoid tumor of the small intestine

In general, tumors of the small intestine (benign or malignant) are rare, much less common than tumors of the colon or stomach. Small carcinoid tumors of the small intestine may not cause any symptoms other than mild abdominal pain. For this reason, it is difficult to determine the presence of a small bowel carcinoid tumor at an early stage, at least until the patient undergoes surgery. It is possible to detect only a small proportion of small intestinal carcinoid tumors in the early stages, and even then this occurs unexpectedly on x-ray. Typically, carcinoid tumors of the small intestine are diagnosed in late stages, when the symptoms of the disease have become apparent and usually after metastases have appeared.

Approximately 10% of carcinoid tumors of the small intestine cause carcinoid syndrome. Typically, the development of carcinoid syndrome means that the tumor is malignant and has reached the liver.

Carcinoid tumors often obstruct the small intestine when they become large. Symptoms of small intestinal obstruction include paroxysmal abdominal pain, nausea and vomiting, and sometimes diarrhea. Obstruction can be caused by two different mechanisms. The first mechanism is an enlargement of the tumor inside the small intestine. The second mechanism is twisting of the small intestine due to fibrosing mesenteritis, a condition caused by a tumor in which extensive scarring occurs in the tissues located in close proximity to the small intestine. Fibrosing mesenteritis sometimes obstructs the arteries that carry blood to the intestines, which can result in the death of part of the intestine (necrosis). In this case, the intestines can rupture, which is a serious threat to life.

Appendiceal carcinoid tumor

Although tumors in the appendix are quite rare, carcinoid tumors are the most common tumors in the appendix (about half of all appendiceal tumors). In fact, carcinoid tumors are found in 0.3% of removed appendixes, but most of them do not grow larger than 1 cm and do not cause any symptoms. In most cases, they are found in appendices that have been removed for reasons other than tumors. Many institutions believe that appendectomy is the most appropriate treatment for these small appendiceal carcinoid tumors. The chances of the tumor returning after an appendectomy are very low. Appendiceal carcinoid tumors larger than 2 cm in 30% can be malignant and form local metastases. Therefore, larger carcinoid tumors should be removed. A simple appendectomy will not help in this case. Fortunately, large carcinoid tumors are quite rare. Carcinoid tumors in the appendix, even with metastases to local tissues, usually do not cause carcinoid syndrome.

Rectal carcinoid tumors

Rectal carcinoid tumors are often diagnosed incidentally during plastic sigmoidoscopy or colonoscopy. Carcinoid syndrome is rare in rectal carcinoid tumors. The likelihood of metastases is related to tumor size; 60-80% chance of metastases for tumors larger than 2 cm. For carcinoid tumors smaller than 1 cm, 2% chance of metastases. In summary, small rectal carcinoid tumors are usually successfully removed, but larger tumors (greater than 2 cm) require extensive surgery, which can even lead to partial removal of the rectum in some cases.

Gastric (stomach) carcinoid tumors

There are 3 types of gastric (stomach) carcinoid tumors: type I, type II and type III.

Type 1 gastric carcinoid tumors are usually less than 1 cm in size and are benign. There are complex tumors that spread throughout the stomach. They usually appear in patients with pernicious anemia or chronic atrophic gastritis (a condition in which the stomach stops producing acid). The lack of acid causes the cells in the stomach, which produce the hormone gastrin, to release large amounts of gastrin, which enters the blood. (Gastrin is a hormone secreted by the body to enhance the activity of stomach acid. Acid in the stomach blocks the production of gastrin. In pernicious anemia or chronic atrophic gastritis, the lack of acid is the result of an increase in the amount of gastrin). In addition, gastrin also influences the transformation of enterochromaffin cells in the stomach into a malignant carcinoid tumor. Treatment for type 1 carcinoid tumors includes treatments such as somatostatin-containing drugs that stop gastrin production or surgical removal of the part of the stomach that produces gastrin.

The second type of gastric carcinoid tumor is less common. Such tumors grow very slowly and the likelihood of them becoming malignant is very low. They appear in patients with a rare genetic disorder called MEN (multiple endocrine neoplasia) type I. In these patients, tumors arise in other endocrine glands, such as the pineal gland, parathyroid gland and pancreas.

The third type of gastric carcinoid tumor are tumors larger than 3 cm that are isolated (appearing one or more at a time) in a healthy stomach. Tumors of the third type are usually malignant and there is a high probability of their deep penetration into the walls of the stomach and the formation of metastases. Type 3 tumors can cause abdominal pain and bleeding, as well as symptoms due to carcinoid syndrome. Type 3 gastric carcinoid tumors usually require surgery to remove the stomach and nearby lymph nodes.

Carcinoid tumors of the colon

Colon carcinoid tumors usually form on the right side of the colon. Like carcinoid tumors of the small intestine, carcinoid tumors of the colon are often discovered at advanced stages. Thus, the average tumor size at diagnosis is 5 cm, and metastases are present in 2/3 of patients. Carcinoid syndrome is rare in carcinoid tumors of the colon.

Symptoms of Carcinoid Syndrome:

Symptoms of carcinoid syndrome vary depending on what hormones the tumors secrete. Typically these are hormones such as serotonin, bradykinin (promotes pain), histamine and chromogranin A.

Typical manifestations of carcinoid syndrome:

• Hyperemia (redness)
• Diarrhea
• Abdominal pain
• Wheezing due to bronchospasm (narrowing of the airways)
• Heart valve damage
• Surgery can cause a complication known as carcinoid crisis.

Hyperemia

Flushing is the most common symptom of carcinoid syndrome. In 90% of patients, hyperemia occurs during the disease. Flushing is characterized by redness or discoloration of the face and neck (or upper body), as well as an increase in temperature. Attacks of hyperemia usually occur suddenly, spontaneously, they can be caused by emotional, physical stress or alcohol consumption. Attacks of hyperemia can last from a minute to several hours. Congestion may be accompanied by a rapid heart rate, low blood pressure, or dizziness if blood pressure drops too much and blood is not getting to the brain. Rarely, hyperemia is accompanied by high blood pressure. The hormones that are responsible for hyperemia are not fully recognized; These may include serotonin, bradykinin and substance P.

Diarrhea

Diarrhea is the second important symptom of carcinoid syndrome. About 75% of patients with carcinoid syndrome experience diarrhea. Diarrhea often occurs along with flushing, but can also occur without it. In carcinoid syndrome, diarrhea is most often caused by serotonin. Medicines that block the action of serotonin, such as ondansetron (Zofran), often relieve diarrhea. Sometimes diarrhea in carcinoid syndrome can occur due to local exposure to a tumor that obstructs the passage of the small intestine.

Heart diseases

Heart disease occurs in 50% of patients with carcinoid syndrome. Carcinoid syndrome usually leads to fibrotic changes in the pulmonary valve of the heart. Impaired valve movement reduces the heart's ability to pump blood from the right ventricle to the lungs and other parts of the body, which can lead to heart failure. Typical symptoms of heart failure include: enlarged liver, swelling of the legs, and fluid accumulation in the abdomen (ascites). The cause of damage to the tricuspid and pulmonary valves of the heart in carcinoid syndrome, in most cases, is intense long-term exposure to serotonin.

Carcinoid crisis

Carcinoid crisis is a dangerous condition that can occur during surgery. A crisis is characterized by a sudden drop in blood pressure, which causes shock. This condition may be accompanied by an excessively rapid heartbeat, elevated blood glucose levels, and severe bronchospasm. Carcinoid crisis can be fatal. The best way to prevent a carcinoid attack is surgery using somatostatin before surgery.

Wheezing

Wheezing occurs in approximately 10% of patients with carcinoid syndrome. Wheezing is a consequence of bronchospasm (spasm of the airways), which occurs as a result of the release of hormones by a carcinoid tumor.

Abdominal pain

Abdominal pain is a common symptom in patients with carcinoid syndrome. Pain may occur due to metastases in the liver, due to the tumor affecting neighboring tissues and organs, or due to intestinal obstruction (read about small intestinal carcinoid tumor below).

Diagnosis of Carcinoid Syndrome:

Diagnosis of carcinoid syndrome confirmed by an increase in the daily urine level of the serotonin metabolite - 5-hydroxyindoleacetic acid.

Treatment of Carcinoid Syndrome:

Treatment of carcinoid syndrome: radical surgical removal of the tumor. For metastases that cannot be removed, therapy is prescribed with octreotide, a long-acting analogue of somatostatin.

For frequent attacks, methyldopa preparations are used (dopegit 0.25-0.5 g 3-4 times a day), opium preparations for diarrhea. It is possible to use prednisolone in a dose of up to 20-30 mg per day.

Which doctors should you contact if you have Carcinoid syndrome:

Is something bothering you? Do you want to know more detailed information about Carcinoid syndrome, its causes, symptoms, methods of treatment and prevention, the course of the disease and diet after it? Or do you need an inspection? You can make an appointment with a doctor– clinic Eurolab always at your service! The best doctors will examine you, study external signs and help you identify the disease by symptoms, advise you and provide the necessary assistance and make a diagnosis. you also can call a doctor at home. Clinic Eurolab open for you around the clock.

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You? It is necessary to take a very careful approach to your overall health. People don't pay enough attention symptoms of diseases and do not realize that these diseases can be life-threatening. There are many diseases that at first do not manifest themselves in our body, but in the end it turns out that, unfortunately, it is too late to treat them. Each disease has its own specific signs, characteristic external manifestations - the so-called symptoms of the disease. Identifying symptoms is the first step in diagnosing diseases in general. To do this, you just need to do it several times a year. be examined by a doctor, in order not only to prevent a terrible disease, but also to maintain a healthy spirit in the body and the organism as a whole.

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Other diseases from the group Oncological diseases:

Carcinoid syndrome is a symptom complex that arises as a result of the release of various hormones by tumors and their entry into the bloodstream. After such an introduction, many readers will decide that they are waiting for a description of another oncological disease: 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 muster one's will into a fist 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 opposite: 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, a tumor is a special case: formidable 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 is a special type of malignant neoplasm that develops 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, also experiences some specific symptoms 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 close the topic of “incurability” of such tumors once and for all, 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: with early diagnosis and timely treatment, a fatal outcome can often be avoided. But to do this, you will have to regularly undergo preventive examinations, give up 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, begin to mourn 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. It may not manifest itself for a long time (in exceptional cases, the problem is revealed by X-ray examination). The diagnosis is usually made after the appearance of metastases, involvement of the liver in the pathological process and, as a result, a significant deterioration in the prognosis. The greatest threat to life is 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 pineal gland.
• 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 site of localization is the right side of the final part of the 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 caused 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 against the background of low 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 rare cases, diarrhea occurs due to a nearby tumor or due to the influence of a metastatic focus.

3. Pathologies of the 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). They are a direct consequence of a spasm of the respiratory tract, but upon a superficial examination they can be explained by problems with the respiratory system.

6. Severe abdominal pain. 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 firsthand with hot flashes, which often appear with the onset of menopause, but when describing carcinoid syndrome, the corresponding clinical signs look slightly 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: the appearance of irregularly shaped bright red spots, 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 of radioactive isotopes of indium-111 followed by visualization on a gamma tomograph);
• endoscopic examination of internal organs using a 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 a 100% effective diagnostic method has not yet been developed. Therefore, even a comprehensive examination guarantees the correct diagnosis only in 70% of cases. But what about the 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 treatment options:

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:

• jejunum and ileum: resection of the affected area, the corresponding part 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 for the generalization of the process, in a complex of therapeutic measures after surgery and subject to the presence of certain unfavorable factors (heart damage, high excretion of 5-HIAA, impaired liver function).

• 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 with an integrated approach aimed at maximizing life extension, it 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 in the early stages often provides a complete cure, so preventive examinations should not be neglected. Believe me, it’s better to spend a few hours a year than to regret missed opportunities later.

ICD-10 code

Carcinoid (carcinoid syndrome) is a rare, potentially malignant, hormonally active tumor originating from argentophilic cells. Most often, carcinoid tumors are localized in the gastrointestinal tract, less often they occur in the bronchi, gall bladder, pancreas and ovaries. The size of tumors is usually not large and varies from 0.1 to 3 cm.

Carcinoid syndrome 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.

Causes

The etiology of carcinoid, like other tumors, is still unclear. The origin of many symptoms of the disease is due to the hormonal activity of the tumor. A significant release of serotonin, lysylbradykinin and bradykinin, histamine, and prostaglandins by tumor cells has been proven.

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.

Classification

One of the most appropriate approaches to the classification of carcinoid tumors of the gastrointestinal tract is their division according to embryogenesis into anterior, middle and posterior. In accordance with this, they distinguish:

• Carcinoid tumors of the anterior part of the digestive tube (bronchi, stomach, duodenum, pancreas); these neoplasms are argentaffin-negative, contain a small amount of serotonin, sometimes secrete 5-hydroxytryptophan and ACTH, and are capable of metastasizing to the bones;
• Carcinoids of the middle part of the digestive tube (jejunum, ileum, right colon) are Argentaffin-positive, contain a lot of serotonin, rarely secrete serotonin or ACTH, rarely metastasize;
• Carcinoid tumors of the posterior part of the digestive tube (transverse colon, descending colon and rectum) are Argentaffin-negative, rarely contain serotonin and ACTH, and can metastasize to bone. Sometimes they are also found in the gonads, prostate, kidneys, mammary glands, thymus gland or skin.

Symptoms

Symptoms and the course of carcinoid consist of local symptoms caused by the tumor itself and the so-called carcinoid syndrome caused by its hormonal activity. Local manifestations are local soreness; Often there are signs reminiscent of acute or chronic appendicitis (with the most common localization of the tumor in the appendix) or symptoms of intestinal obstruction, intestinal bleeding (if localized in the small or large intestine), pain during defecation and discharge of scarlet blood with feces (with rectal carcinoid ), weight loss, anemia. Carcinoid syndrome includes peculiar vasomotor reactions, attacks of bronchospasm, hyperperistalsis of the gastrointestinal tract, characteristic skin changes, lesions of the heart and pulmonary artery. It is not observed in a pronounced form in all patients, but more often with tumor metastases to the liver and other organs, especially multiple ones.

Typical symptoms are:

• Hyperemia
• Diarrhea
• Abdominal pain
• Wheezing due to bronchospasm (narrowing of the airways)
• Heart valve disease
• Surgery can cause a complication known as carcinoid crisis.

Flushing is the most common symptom of carcinoid syndrome. In 90% of patients, hyperemia occurs during the disease. Flushing is characterized by redness or discoloration of the face and neck (or upper body), as well as an increase in temperature. Flushing attacks usually occur suddenly and spontaneously, but they can also be caused by emotional stress, physical stress or alcohol consumption.

Attacks of hyperemia can last from a minute to several hours. Congestion may be accompanied by a rapid heart rate, low blood pressure, or dizziness if blood pressure drops too much and blood is not getting to the brain. Rarely, hyperemia is accompanied by high blood pressure. The hormones that are responsible for hyperemia are not fully recognized.

Diarrhea is the second important symptom of carcinoid syndrome. About 75% of patients with carcinoid syndrome experience diarrhea. Diarrhea often occurs along with flushing, but can also occur without it. In carcinoid syndrome, diarrhea is most often caused by serotonin.

Heart disease occurs in 50% of patients with carcinoid syndrome. Carcinoid syndrome usually results in scarring and stiffness of the pulmonary valve on the right side of the heart. Stiffness of these two valves reduces the heart's ability to pump blood from the right ventricle to the lungs and other parts of the body, which can lead to heart failure.

Typical symptoms of heart failure include an enlarged liver (due to the supply of blood to the heart in heart failure when it is unable to pump all the incoming blood), swelling of the legs and ankles (dropsy), and swelling of the abdomen due to fluid buildup (abdominal hydrops). The cause of damage to the tricuspid and pulmonary valves of the heart in carcinoid syndrome, in most cases, is the intense long-term effect of serotonin on the blood.

Carcinoid attack is a dangerous condition that can occur during surgery. It is characterized by a sudden drop in blood pressure that causes shock, sometimes accompanied by an excessively rapid heart rate, increased blood glucose, and severe bronchospasm.

A carcinoid attack can be fatal. The best way to prevent a carcinoid attack is surgery.

Wheezing occurs in approximately 10% of patients with carcinoid syndrome. Wheezing is a consequence of bronchospasm (spasm of the airways), which occurs as a result of the release of hormones by a carcinoid tumor.

Abdominal pain is a common symptom in patients with carcinoid syndrome. Pain may occur due to metastases in the liver, due to the fact that the tumor affects neighboring tissues and organs, or intestinal obstruction.

Diagnostics

The diagnosis is confirmed by a high level of sorotonin in the blood plasma, increased urinary excretion of 5-hydroxyindoleacetic acid, as well as the detection of intestinal, liver, and lung tumors during instrumental research. Biochemical parameters 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 by intestinal obstruction, when the excretion of 5-hydroxyindoleacetic acid may increase to 9–25 mg/day.

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

Comprehensive topical diagnosis of 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.

Treatment

Considering the general principles of therapy for carcinoid syndrome, it should be noted that the earliest possible removal of the tumor is advisable. 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 is 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 the 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 cyproheptadine and deseryl are the most widely used. Cyproheptadine has not only an antiserotonin, but also an antihistamine effect. It is prescribed intravenously in a dose of 6 to 40 mg. Deseryl is used orally at a dose of 6 to 24 mg and intravenously over 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 is 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.

Thus, the main type of treatment for patients with carcinoid and neuroendocrine tumors is surgery. Their drug treatment is based on chemotherapy, the use of somatostatin analogues and α-interferons.

– a complex of symptoms that arise as a result of the hormonal activity of tumors from cells of the APUD system. Includes hot flashes, persistent diarrhea, fibrous lesions of the right side of the heart, abdominal pain and bronchospasm. During surgical interventions, a life-threatening carcinoid crisis may develop. Carcinoid syndrome is diagnosed on the basis of anamnesis, complaints, objective examination data, results of determining the level of blood hormones, CT, MRI, scintigraphy, radiography, endoscopy and other studies. Treatment is surgical removal of the tumor, drug therapy.

Carcinoid syndrome is a symptom complex observed in potentially malignant, slow-growing neuroendocrine tumors that can arise in almost any organ. In 39% of cases, neoplasms that provoke carcinoid syndrome are localized in the small intestine, in 26% - in the appendix, in 15% - in the rectum, in 1-5% - in other parts of the large intestine, in 2-4% - in the stomach , 2-3% - in the pancreas, 1% - in the liver and 10% - in the bronchi. In 20% of cases, carcinoid tumors of the gastrointestinal tract are combined with other neoplasms of the large intestine. Carcinoid syndrome usually develops between the ages of 50 and 60, and men and women are equally affected.

Carcinoid was first described at the end of the 19th century. In the 50s of the last century, a group of researchers led by Waldenström compiled an extensive description of the histological and histochemical features of carcinoid tumors, and also proved the connection between such neoplasms and the development of carcinoid syndrome. For a long time, carcinoids were considered benign tumors, but research in recent years has shaken this point of view. It was found that, despite their slow growth, the formations have certain signs of malignancy and can metastasize to distant organs. Treatment of carcinoid syndrome is carried out by doctors working in the field of oncology, endocrinology, gastroenterology, cardiology, general surgery and other specialties.

Causes of carcinoid syndrome

The cause of the development of carcinoid syndrome is the hormonal activity of tumors originating from the neuroendocrine cells of the APUD system. Symptoms are determined by changes in the levels of various hormones in the patient's blood. Typically, such neoplasms secrete large amounts of serotonin. As a rule, there is an increase in the production of histamine, prostaglandins, bradykinin and polypeptide hormones.

Carcinoid syndrome does not occur in all patients with carcinoid tumors. In carcinoids of the small and large intestine, this syndrome usually develops only after the appearance of metastases in the liver. This is explained by the fact that hormones from the intestines with the blood enter the liver through the portal vein system, and are then destroyed by liver enzymes. Metastasis to the liver leads to the fact that the products of tumor metabolism begin to directly enter the general bloodstream through the hepatic veins, without being broken down in liver cells.

With carcinoids localized in the lungs, bronchi, ovaries, pancreas and other organs, carcinoid syndrome can develop before the onset of metastasis, since the blood from these organs does not pass through the portal vein system before entering the systemic circulation and is not “purified” in the liver tissue . Obviously malignant neoplasms cause carcinoid syndrome more often than tumors that do not show signs of malignancy.

An increase in serotonin levels provokes diarrhea, abdominal pain, malabsorption and cardiac pathology. As a rule, with carcinoid syndrome, fibrous degeneration of the endocardium of the right half of the heart is observed. The left side of the heart is rarely affected because serotonin is destroyed as blood passes through the lungs. An increase in bradykinin and histamine causes hot flashes. The role of polypeptide hormones and prostaglandins in the development of carcinoid syndrome has not yet been clarified.

Symptoms of carcinoid syndrome

The most common manifestation of carcinoid syndrome is hot flashes. This symptom is observed in 90% of patients. There is a sudden, periodic, paroxysmal redness of the upper half of the body. Typically, hyperemia is especially pronounced in the face, back of the head and neck. Patients with carcinoid syndrome complain of sensations of heat, numbness and burning. Hot flashes are accompanied by increased heart rate and a drop in blood pressure. Dizziness may occur due to decreased blood supply to the brain. During attacks, redness of the sclera and lacrimation may occur.

In the initial stages of carcinoid syndrome, hot flashes appear once every few days or weeks. Subsequently, their number gradually increases to 1-2 or even 10-20 times a day. The duration of hot flashes in carcinoid syndrome can range from 1-10 minutes to several hours. Typically, attacks occur due to consumption of alcohol, spicy, fatty and spicy foods, physical activity, psychological stress, or taking medications that increase serotonin levels. Less commonly, hot flashes develop spontaneously, without any apparent reason.

Diarrhea is detected in 75% of patients with carcinoid syndrome. It occurs due to increased motility of the small intestine under the influence of serotonin. It is chronic and persistent. The severity of the symptom can vary greatly. Due to malabsorption, all types of metabolism (protein, carbohydrate, fat, water-electrolyte) are disrupted, and hypovitaminosis develops. With long-term carcinoid syndrome, drowsiness, muscle weakness, fatigue, thirst, dry skin and weight loss are noted. In severe cases, swelling, osteomalacia, anemia and pronounced trophic changes in the skin occur due to gross metabolic disorders.

Cardiac pathology is found in half of patients suffering from carcinoid syndrome. As a rule, endocardial fibrosis is detected, accompanied by damage to the right half of the heart. Fibrous changes cause incompetence of the tricuspid and pulmonary valves and provoke pulmonary stenosis. Valve failure and pulmonary artery stenosis in carcinoid syndrome can lead to the development of heart failure and stagnation in the systemic circulation, which are manifested by edema of the lower extremities, ascites, pain in the right hypochondrium due to liver enlargement, swelling and pulsation of the veins of the neck.

In 10% of patients with carcinoid syndrome, bronchospasm is observed. Patients are bothered by attacks of expiratory shortness of breath, accompanied by whistling and buzzing wheezing. Another fairly common manifestation of carcinoid syndrome is abdominal pain, which can be caused by a mechanical obstruction to the movement of intestinal contents due to the growth of the primary tumor or the occurrence of secondary lesions in the abdominal cavity.

A dangerous complication of carcinoid syndrome can be carcinoid crisis - a condition that occurs during surgical interventions, accompanied by a sharp decrease in blood pressure, increased heart rate, severe bronchospasm and an increase in blood glucose levels. The shock that occurs during a carcinoid crisis poses an immediate threat to the patient's life and can lead to death.

Diagnosis of carcinoid syndrome

The diagnosis is made by an oncologist based on characteristic symptoms, external examination data and objective studies. An increased level of serotonin is detected in the blood plasma. A high content of 5-hydroxyindoleacetic acid is detected in the urine. To exclude a false positive result, for 3 days before testing, a patient with suspected carcinoid syndrome is advised to refrain from taking foods containing large amounts of serotonin (walnuts, eggplants, avocados, tomatoes, bananas, etc.) and certain medications.

In doubtful cases, tests with alcohol, catecholamines or calcium gluconate are performed to stimulate hot flashes. To determine the location of the tumor and identify metastases, a patient with carcinoid syndrome is referred to CT and MRI of internal organs, scintigraphy, radiography, gastroscopy, colonoscopy, bronchoscopy and other studies. If there are sufficient indications, laparoscopy can be used. Carcinoid syndrome is differentiated from systemic mastocytosis, hot flashes during menopause and side effects when taking certain medications.

Treatment of carcinoid syndrome

The main treatment method is surgery. Depending on the location and extent of the process, radical excision of the primary lesion or various palliative operations are possible. For carcinoid syndrome caused by tumors of the small intestine, resection of the small intestine along with the mesentery is usually performed. In case of damage to the large intestine and neoplasms located in the area of ​​the ileocecal sphincter, a right hemicolectomy is performed.

For carcinoid syndrome caused by a tumor in the appendix, an appendectomy is performed. Some oncologists additionally remove nearby lymph nodes. Palliative surgical interventions are indicated at the stage of metastasis and involve the removal of large lesions (both secondary and primary) to reduce hormone levels and reduce the severity of carcinoid syndrome. Another way to reduce the manifestations of carcinoid syndrome is embolization or ligation of the hepatic artery.

The indication for chemotherapy for carcinoid syndrome is the presence of metastases. In addition, this treatment method is used in the postoperative period for heart damage, liver dysfunction and high levels of 5-hydroxyindoleacetic acid in the urine. To avoid the development of a carcinoid crisis, treatment begins with small doses of drugs, gradually increasing the dosage. The effectiveness of chemotherapy for carcinoid syndrome is low. Significant improvement is observed in no more than 30% of patients, the average duration of remission is 4-7 months.

As symptomatic therapy for carcinoid syndrome, serotonin antagonist drugs are prescribed: fluoxetine, sertraline, paroxetine, etc. For tumors that produce large amounts of histamine, diphenhydramine, ranitidine and cimetidine are used. To eliminate diarrhea, it is recommended to take loperamide. In addition, for carcinoid syndrome, lanreotide and octreotide are widely used, which suppress the secretion of biologically active substances in the area of ​​the tumor and can significantly reduce the manifestations of this syndrome in more than half of the patients.

The prognosis for carcinoid syndrome is determined by the extent and grade of the tumor. With radical removal of the primary lesion and the absence of metastases, complete recovery is possible. With metastasis, the prognosis worsens. Due to the slow progression of carcinoid, life expectancy in such cases ranges from 5 to 15 years or more. The cause of death is multiple metastases to distant organs, exhaustion, heart failure or intestinal obstruction.