Carcinoid syndrome: signs, diagnosis and treatment

Medical statistics show that the number of oncological diseases is steadily increasing. Fatal outcomes are registered with increasing frequency every year. This fact causes more and more anxiety and forces doctors to conduct preventive work among the population with a view to at least a little to restrain such aggressive expansive growth.

Carcinoid Syndrome: What is it?

Carcinoids are the most common tumors from the cells of the neuroendocrine system. These cells can be found in any organ and tissue of the body. Their main function is the production of highly active protein substances. About eighty percent of all carcinoids are located in the gastrointestinal tract, in second place are the lungs. Much less often, in only 5 percent of cases, neoplasms are found in other organs and tissues.

Carcinoid syndrome and carcinoid tumor are inseparable, since the syndrome is a set of symptoms that appear against the background of growth and activity of malignant formation. After all, the body reacts to the ingestion of hormones secreted by the tumor into the bloodstream. Carcinoids of the intestine can be "dumb", that is, do not manifest themselves until the moment when there are metastases and the disease does not go to the terminal stage.

Causes

The researchers do not yet have enough information to understand why carcinoid syndrome and carcinoid tumors develop. Scientists can build hypotheses about the etiology of the disease, but all agree that the immune system at some point ceases to recognize the mutated cells of the APUD system (the acronym of the first letters of the words "amines," "precursor," "assimilation," "decarboxylation" ).

It is enough for one malignant cell to develop a full-fledged neoplasm that will produce hormones and change all biochemical processes in the human body for its own needs.

Pathogenesis

How does the carcinoid syndrome develop? What is it and how can it be prevented? Given that the neuroendocrine cells are located throughout the body, and the clinical manifestations of the tumor is almost not given, doctors can not stop this process.

At one point in the cell there is a "breakage" of DNA, which is incorrectly restored or continues to function with the damaged fragment. This leads to a disruption in the function of the cell and its uncontrolled division. If the body is healthy, then the immune system will react to the mutation and get rid of the suspicious element. If this does not happen, the cell will multiply, create millions of copies of itself and begin to spread toxic substances and hormones throughout the body.

Often doctors are unable to detect the location of the tumor, despite the clinically expressed carcinoid syndrome.

Carcinoma in the lungs

Only in ten percent of cases in the respiratory system can develop a tumor and, accordingly, a carcinoid syndrome. Signs in the lung will be nonspecific, and sometimes they will not be at all. This is due to the relatively small size of the neoplasm and the lack of metastasis. Patients turn to help with already started disease and, as a rule, not to the oncologist, but first to the therapist. He can long and unsuccessfully treat bronchiolitis, asthma or respiratory failure, until he suspects the presence of an oncological process.

Symptoms in this case are atypical:

• Rapid, rapid heartbeat;
• Dyspeptic phenomena;
• A feeling of heat and a rush of blood to the upper half of the trunk;
• Cough, shortness of breath;
• Bronchospasm.

With this set of disorders, it is difficult to suspect a carcinoid. There is no exhaustion, a sharp weight loss, a decrease in immunity, fatigue and other characteristic symptoms of the oncological process.

Tumor of the small intestine

In the small intestine, a tumor and accompanying carcinoid syndrome are registered more often than in the lungs. His symptoms are very scarce. Often there are only nonspecific abdominal pains. This is due to the small size of the tumor. Sometimes it can not be detected even on an operation. Often, education is noticed accidentally during an X-ray study.

Only about ten percent of all small bowel tumors of this etiology are the cause of carcinoid syndrome. For the doctor, this means that the process became malignant and spread to the liver. Such tumors can cause obturation of the lumen of the intestine and, as a consequence, intestinal obstruction. The patient enters the hospital with cramping pains, nausea, vomiting and stool disorders. And the cause of this state is known only on the operating table.

Obstruction can be caused both directly by the size of the tumor, and the torsion of the intestine due to the phenomena of fibrosis and inflammation of its mucous membrane. Sometimes scars disrupt the blood supply of the intestinal tract, which leads to necrosis and peritonitis. Any of these conditions is life-threatening and can lead to the patient's death.

Tumor of the appendix

Tumors of an appendix are a rare phenomenon in itself. Among them, carcinoids occupy an honorable first place, but practically do not cause carcinoid syndrome. Their signs are extremely scarce. As a rule, these are the findings of pathologists after appendectomy. The tumor in size does not reach one centimeter and behaves very "quietly". The chances that after the removal of the appendix the tumor will appear in some other place, insignificant.

But if the detected tumor was two centimeters or more in size, then one should beware of metastasizing into local lymph nodes and dissemination of tumor cells to other organs. Ordinary appendectomy in this case will not cope with all screenings, and it will be necessary to attract oncologists for complex treatment.

Rectal carcinoid tumor

Another localization, in which the carcinoid syndrome is practically not expressed. There are no signs and tumors are accidentally found during diagnostic manipulation, such as a colonoscopy or sigmoidoscopy.

The likelihood of malignancy (malignancy) and the appearance of distant metastases depends on the size of the tumor. If its diameter exceeds two centimeters, the risk of complications is about eighty percent. If the neoplasm does not reach even one centimeter in diameter, then you can be confident at ninety-eight percent that there is no metastasis.

Therefore, the approach to treatment in these two cases will be different. A small tumor is usually removed by economical gut resection, and if there are signs of malignancy, then removal of the entire rectum, as well as chemotherapy, is required.

Gastric carcinoid

There are three types of carcinoid stomach tumors that cause carcinoid syndrome. Symptoms of tumors of the first type:

• Small sizes (up to 1 cm);
• Benign course.

Perhaps a complex spread of the tumor, when the process captures the entire stomach. They are associated with percinosis anemia or chronic gastritis in the patient. Treatment of such tumors consists of taking somatostatin, suppressing the production of gastrin or resection of the stomach.

Tumors of the second type grow slowly, extremely rarely become malignant. They prevail in patients with a genetic disorder, such as multiple endocrine neoplasia. Not only the stomach, but also the epiphysis, thyroid gland, pancreas can be affected.

The third type of tumors are large neoplasms growing in a healthy stomach. They are malignant, penetrate deep into the organ wall and give multiple metastases. May cause perforation and bleeding.

Tumors of the large intestine

The large intestine is the place where the carcinoid syndrome and the carcinoid tumor are most often diagnosed. A photo of the mucosa in the examination of this segment of the intestine shows the presence of large (five centimeters or more) tumors. They metastasize into regional lymph nodes and are almost always malignant.

Oncologists in such cases advise a radical operation with adjuvant and neoadjuvant chemotherapy to achieve a better effect. But the survival prognosis for these patients is still unfavorable.

Carcinoid Syndrome: symptoms, photos

Manifestations of carcinoid syndrome depend on what substances the tumor secretes. These can be serotonin, bradykinin, histamine or chromogranin A. The most typical signs of the disease are:

• Redness of the skin of the face and upper half of the trunk. It occurs in almost all patients. It is accompanied by a local increase in temperature. Attacks occur spontaneously, can be triggered by alcohol intake, stress, or physical exertion. There is a tachycardia, pressure drops.
• Stool disorder. It is present in ¾ patients. As a rule, this is due to irritation of the mucosa of the digestive tract, violation of the permeability of the intestine.
• Dysfunction of the heart is inherent in half the patients. Carcinoid syndrome promotes the formation of stenoses of the heart valves and causes heart failure.
• Chryps in the lungs are a consequence of spasm of the bronchi.
• Pain in the abdomen is associated with the appearance of metastases in the liver, intestinal obstruction or tumor germination in other organs.

The carcinoid crisis is a condition characterized by a sharp drop in pressure during the surgical intervention. Therefore, patients are assigned somatostatin before similar manipulations.

Diagnostics

Is it possible to detect a carcinoid syndrome? Signs, photos, treatment of disparate symptoms does not give a clear clinical picture, or visible results. Most often, a tumor is an accidental finding of a surgeon or radiologist. In order to confirm the presence of a tumor, it is necessary to make a biopsy of the affected area and to examine the tissues.

You can still use tests to identify elevated levels of hormones, but these data can indicate several diseases, and the doctor has yet to figure out which of them he is dealing with. The most specific test is the amount of 5-hydroxyindoleacetic acid. If its level is raised, then the probability of carcinoid is almost 90%.

Chemotherapy

Does it make sense to chemotherapy treat carcinoid syndrome? Symptoms, photos of the mucosa in the FGDs, a biopsy of regional lymph nodes and changes in the hormonal background can give the doctor an idea of the complications that caused the presence of the tumor in the patient's body. If the prognosis for the patient is unfavorable even in the case of tumor removal, then experts advise to resort to chemotherapy.

Most often, doctors use cytostatics to suppress the growth and development of tumors. But because of the large number of side effects this method is recommended to use only in case of emergency. In addition, its effectiveness is only 40%.

Symptomatic treatment

There are uncomfortable sensations that are observed against the background of pathologies such as carcinoid syndrome and carcinoid tumors. Symptoms are usually closely related to the type of hormone that secretes the tumor. If it is serotonin, then the patient is prescribed antidepressants. If the main "aggressor" is histamine, then the blockers of histamine receptors go to the forefront of therapy.

Synthetic analogues of somatostatin were found, reducing symptomatology by almost 90%. They suppress the production of several hormones at once and thus provide themselves with a firm place in the therapy of this disease.

Operative treatment

An important stage of treatment is removal of the tumor from the body, search and resection of metastases, ligation of the arteries of the liver.

The amount of surgery affects the location and size of the tumor, the presence or absence of metastases. Most often, surgeons remove the affected part of the organ and packets of regional lymph nodes. As a rule, this is enough to permanently rid the person of carcinoid. In neglected cases, when radical treatment is impossible, the patient is offered to perform embolicization of the hepatic artery to remove the symptoms of the disease.

Forecast

What can patients with a diagnosis of carcinoid syndrome and carcinoid tumors expect? Tides, attacks of rapid heart rate and shortness of breath, most likely, remain with them until the end of life, but become less pronounced after treatment.

There are cases when patients lived more than ten years after the operation and symptomatic therapy. But the average life expectancy is about 5-10 years. The worst forecast is for tumors located in the lungs, and the best is in the appendix.