Medical statistics show that the number of oncological diseases is growing steadily. Fatal outcomes are recorded more and more every year. This fact causes more and more concern and forces doctors to carry out preventive work among the population in order to at least contain such aggressive expansive growth.
Carcinoid syndrome: what is it?
Carcinoids are the most common tumors from 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, followed by the lungs. Much less often, in only five 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 the malignant formation. After all, the body reacts to the ingestion of hormones secreted by the tumor into the blood. Intestinal carcinoids can be "dumb", that is, do not manifest themselves in any way until metastases appear and the disease passes into the terminal stage.
Causes
Researchers do not have enough information to understand why carcinoid syndrome and carcinoid tumor develop. Scientists can hypothesize about the etiology of the disease, but everyone agrees that the immune system at some point ceases to recognize mutated cells of the APUD system (acronym from the first letters of the words "amines", "predecessor", "assimilation", "decarboxylation" )
One malignant cell is enough for a full-fledged neoplasm to develop from it, which will produce hormones and change all biochemical processes in the human body to fit its own needs.
Pathogenesis
How does carcinoid syndrome develop? What is it and how to prevent it? Given that neuroendocrine cells are located throughout the body, and the tumor practically does not give clinical manifestations, doctors are not able to stop this process.
At one point in the cell there is a “breakdown” of DNA, which is incorrectly restored or continues to function with a damaged fragment. This leads to impaired cell function and its uncontrolled division. If the body is healthy, then the immune system will respond to the mutation and get rid of the suspicious element. If this does not happen, then the cell will multiply, create millions of copies of itself and begin to spread toxic substances and hormones throughout the body.
Often, doctors are not able to identify the location of the tumor, despite the clinically pronounced carcinoid syndrome.
Lung carcinoma
Only in ten percent of cases in the respiratory system can a tumor develop and, accordingly, 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 seek help already with a running disease and, as a rule, not to an oncologist, but first to a therapist. He can treat bronchiolitis, asthma or respiratory failure for a long time and without success, until he suspects the presence of an oncological process.
Symptoms in this case are atypical:
- rapid, strong heartbeat;
- dyspeptic symptoms;
- a feeling of heat and flushing of the upper half of the body;
- cough, shortness of breath;
- bronchospasm.
With this set of disorders, it is difficult to suspect a carcinoid. There is no exhaustion, drastic weight loss, decreased immunity, fatigue and other characteristic symptoms of the oncological process.
Small intestine tumor
In the small intestine a little more often than in the lungs, the tumor and the accompanying carcinoid syndrome are recorded. Its signs are very scarce. Often there are only non-specific abdominal pains. This is due to the small size of the tumor. Sometimes it cannot be detected even during operations. Often, education is noticed by chance during an X-ray examination.
Only about ten percent of all small intestine tumors of this etiology cause carcinoid syndrome. For the doctor, this means that the process has become malignant and has spread to the liver. Such tumors can cause obstruction of the intestinal lumen and, as a result, intestinal obstruction. The patient is admitted to the hospital with cramping pains, nausea, vomiting and stool disorders. And the reason for this condition is found out only on the operating table.
Obstruction can be caused both directly by the size of the tumor, and torsion of the intestine due to the phenomena of fibrosis and inflammation of its mucous membrane. Sometimes scars disrupt the blood supply to the intestinal tract, which leads to necrosis and peritonitis. Any of these conditions is life threatening and can lead to the death of the patient.
Appendix tumor
Appendix tumors are a rare occurrence in and of themselves. 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 findings of pathologists after appendectomies. The tumor in size does not reach even one centimeter and behaves very "quietly." The chances that after the removal of the appendix the tumor will appear in some other place are insignificant.
But if the detected neoplasm was two centimeters or more in size, then metastasis to local lymph nodes and dissemination of tumor cells to other organs should be feared. Ordinary appendectomy in this case will not cope with all screenings, and oncologists will have to be involved for complex treatment.
Rectal carcinoid tumor
Another localization in which carcinoid syndrome is practically not expressed. There are no signs and tumors are accidentally found during diagnostic procedures, such as 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, then the risk of complications is about eighty percent. If the neoplasm does not reach one centimeter in diameter, then you can be sure of ninety-eight percent that there are no metastases.
Therefore, the approach to treatment in these two cases will be different. A small tumor is usually removed by an economical bowel resection, and if there are signs of malignancy, then removal of the entire rectum and chemotherapy will be required.
Gastric carcinoid
There are three types of carcinoid tumors of the stomach that cause carcinoid syndrome. Signs of tumors of the first type:
- small sizes (up to 1 cm);
- benign course.
A complex spread of the tumor is possible when the process captures the entire stomach. They are associated with pernicious anemia or chronic gastritis in a patient. The treatment of such tumors consists in taking somatostatins, inhibiting the production of gastrin or gastric resection.
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 can be affected, but also the pineal gland, thyroid, pancreas.
The third type of tumor is a large tumor that grows in a healthy stomach. They are malignant, penetrate deep into the wall of the organ and give multiple metastases. May cause perforation and bleeding.
Colon tumors
The large intestine is the place where the most commonly diagnosed are carcinoid syndrome and carcinoid tumor. A photo of the mucosa during examination of this segment of the intestine shows the presence of large (five centimeters or more) tumors. They metastasize to regional lymph nodes and are almost always malignant.
Oncologists in such cases advise radical surgery with adjuvant and neoadjuvant chemotherapy to achieve a better effect. But the survival prognosis for these patients is still poor.
Carcinoid syndrome: symptoms, photo
Manifestations of carcinoid syndrome depend on what substances the tumor secretes. It 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 body. It occurs in almost all patients. Accompanied by local fever. Attacks occur spontaneously, can be triggered by the intake of alcohol, stress or physical exertion. In this case, tachycardia is observed, pressure drops.
- Upset stool. Present in ¾ patients. As a rule, this is due to irritation of the mucosa of the digestive tract, impaired intestinal patency.
- Disorders of the heart are inherent in half of the patients. Carcinoid syndrome contributes to the formation of stenosis of the heart valves and causes heart failure.
- Wheezing in the lungs is the result of bronchospasm.
- Abdominal pain is associated with the appearance of metastases in the liver, intestinal obstruction, or tumor invasion into other organs.
A carcinoid crisis is a condition that is characterized by a sharp drop in pressure during surgery. Therefore, patients are prescribed somatostatin before such manipulations.
Diagnostics
Can carcinoid syndrome be detected? Signs, photos, treatment of disparate symptoms does not give a clear clinical picture, nor visible results. Most often, a tumor is an accidental find by a surgeon or radiologist. In order to confirm the presence of a neoplasm, you need to do a biopsy of the affected area and examine the tissue.
You can still use tests to detect elevated levels of hormones, but these data may indicate several diseases, and the doctor has yet to figure out which one he is dealing with. The most specific is the test for the amount of 5-hydroxyindoleacetic acid. If its level is increased, then the likelihood of a carcinoid is almost 90%.
Chemotherapy
Does it make chemotherapy to treat carcinoid syndrome? Signs, a photo of the mucous membrane during FGDS, a regional lymph node biopsy and changes in the hormonal background can give the doctor an idea of the complications caused by the presence of a tumor in the patient’s body. If the prognosis for the patient is unfavorable even in the case of removal of the tumor, then experts advise resorting to chemotherapy.
Most often, doctors use cytostatics to suppress the growth and development of tumors. But due to the large number of side effects, this method is recommended to be used 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 such pathologies as carcinoid syndrome and carcinoid tumor. Symptoms are usually closely related to the type of hormone that a tumor secretes. If it is serotonin, then antidepressants are prescribed to the patient. If the main "aggressor" is histamine, then histamine receptor blockers come to the fore in therapy.
Synthetic analogues of somatostatin were found that reduce symptoms by almost 90%. They suppress the production of several hormones at once and thereby secure a solid place in the treatment of this disease.
Surgical treatment
An important stage of treatment is the removal of a tumor from the body, the search and resection of metastases, and ligation of the arteries of the liver.
The location and size of the neoplasm, the presence or absence of metastases affects the volume of surgical intervention. Most often, surgeons remove the affected part of the organ and packages of regional lymph nodes. As a rule, this is enough to permanently rid a person of a carcinoid. In advanced cases, when radical treatment is impossible, the patient is offered to carry out embolization of the hepatic artery to remove the symptoms of the disease.
Forecast
What should patients with a diagnosis of carcinoid syndrome and carcinoid tumor expect? Hot flashes, bouts of heart palpitations and shortness of breath are likely to remain with them until the end of life, but will become less pronounced after treatment.
Medicine knows cases when patients lived more than ten years after surgery and symptomatic therapy. But on average, their life expectancy is about 5-10 years. Tumors located in the lungs have the worst prognosis, and the appendix has the best prognosis.