Kartsinoidny syndrome – a complex of the symptoms resulting from hormonal activity of tumors from APUD-system cages. Includes inflows, persistiruyushchy diarrhea, fibrous defeat of the right half of heart, belly-ache and a bronchospasm. When performing surgeries development of life-threatening kartsinoidny crisis is possible. The Kartsinoidny syndrome is diagnosed on the basis of the anamnesis, complaints, the yielded objective survey, results of determination of level of hormones of blood, KT, MPT, a stsintigrafiya, a X-ray analysis, endoscopy and other researches. Treatment – surgical removal of a tumor, medicamentous therapy.
Kartsinoidny syndrome – the simptomokompleks which is observed at potentially malignant slowly growing neuroendocrine tumors which can arise practically in any bodies. In 39% of cases of a new growth provoking a kartsinoidny syndrome are localized in a small intestine, in 26% - in an appendix, in 15% - in a rectum, in 1-5% - in other departments of a large intestine, in 2-4% - in a stomach, in 2-3% - in a pancreas, in 1% - in a liver and in 10% - in bronchial tubes. In 20% of cases kartsinoidny tumors of a GIT are combined with other new growths of a large intestine. The Kartsinoidny syndrome usually develops at the age of 50-60 years, men and women suffer equally often.
Kartsinoid was for the first time described at the end of the 19th century. In the 50th years of the last century the group of researchers under the leadership of Valdenstrem made the expanded description of histologic and histochemical features of kartsinoidny tumors, and also proved communication between such new growths and development of a kartsinoidny syndrome. For a long time kartsinoida were considered as benign tumors, however researches of the last years shook this point of view. It was established that, despite slow growth, educations possess certain signs of a zlokachestvennost and can give metastasises in the remote bodies. Treatment of a kartsinoidny syndrome is performed by the doctors working in the field of oncology, endocrinology, gastroenterology, cardiology, the general surgery and other specialties.
Reasons of a kartsinoidny syndrome
Hormonal activity of the tumors coming from neuroendocrine cages of APUD-system becomes the reason of development of a kartsinoidny syndrome. Symptoms are defined by change of level of various hormones in the patient's blood. Usually such new growths allocate a large amount of serotonin. As a rule, increase in production of a histamine, prostaglandins, a bradikinin and polypeptide hormones is observed.
The Kartsinoidny syndrome arises not at all patients with kartsinoidny tumors. At kartsinoida of a small and thick intestine this syndrome usually develops only after emergence of metastasises in a liver. This results from the fact that hormones from intestines with blood come to a liver through system of a vorotny vein, and then collapses hepatic enzymes. Metastasis in a liver leads to the fact that products of tumoral metabolism begin to come directly to the general blood-groove through hepatic veins, without being exposed to splitting in cells of a liver.
At the kartsinoida which are localized in lungs, bronchial tubes, ovaries, a pancreas and other bodies, the kartsinoidny syndrome can develop prior to the beginning of metastasis as blood from these bodies before receipt in a system blood-groove does not pass through system of a vorotny vein and is not exposed to "cleaning" in liver tissue. Obviously malignant new growths cause a kartsinoidny syndrome more often than the tumors which are not showing malignization signs.
Increase in level of serotonin provokes diarrhea, belly-aches, malabsorption and cardiac pathology. As a rule, at a kartsinoidny syndrome fibrous regeneration of an endokard of the right half of heart is observed. The left half of heart is surprised seldom as serotonin collapses when passing blood through lungs. Increase in quantity of a bradikinin and histamine causes inflows. The role of polypeptide hormones and prostaglandins in development of a kartsinoidny syndrome is not found out yet.
Symptoms of a kartsinoidny syndrome
The most widespread manifestation of a kartsinoidny syndrome are inflows. This symptom is observed at 90% of patients. Sudden, periodic, pristupoobrazny reddening of the top half of a trunk is noted. Usually hyperaemia is especially brightly expressed in a face, a nape and a neck. Patients with a kartsinoidny syndrome show complaints to feeling of heat, a sleep and burning. Inflows are followed by increase of warm reductions and falling of arterial pressure. The dizzinesses caused by deterioration in blood supply of a brain are possible. During attacks can be observed reddening a skler and dacryagogue.
At the initial stages of development of a kartsinoidny syndrome inflows appear once in several days or weeks. In the subsequent their quantity gradually increases to 1-2 or even 10-20 times a day. Duration of inflows at a kartsinoidny syndrome can fluctuate of 1-10 minutes till several o'clock. Usually attacks arise against the background of alcohol intake, spicy, greasy and spicy food, physical activity, a psychological stress or reception of the medicines promoting increase in level of serotonin. Less often inflows develop spontaneously, without any visible reasons.
Diarrhea comes to light at 75% of patients with a kartsinoidny syndrome. Arises because of strengthening of motility of a small intestine under the influence of serotonin. Has chronic persistiruyushchy character. Expressiveness of a symptom can strongly differ. Because of malabsorption all types of exchange are broken (proteinaceous, carbohydrate, fatty, water and electrolytic), hypovitaminoses develop. At it is long the current kartsinoidny syndrome drowsiness, weakness of muscles, bystry fatigue, thirst, dryness of integuments and weight reduction are noted. In hard cases there are hypostases, an osteomalyation, anemia and the expressed trophic changes of integuments caused by rough exchange frustration.
Cardiac pathology is found in a half of the patients suffering from a kartsinoidny syndrome. As a rule, the endokardialny fibrosis which is followed by defeat of the right half of heart comes to light. Fibrous changes become the reason of insolvency of three-leaved and pulmonary valves and provoke a stenosis of a pulmonary trunk. Insolvency of valves and a stenosis of a pulmonary artery at a kartsinoidny syndrome can cause development of heart failure and stagnation in a big circle of blood circulation which are shown by hypostases of the lower extremities, ascites, pains in the right podreberye because of increase in a liver, swelling and a pulsation of veins of a neck.
At 10% of patients with a kartsinoidny syndrome the bronchospasm phenomena are observed. Patients are disturbed by the attacks of expiratory short wind which are followed by the whistling and buzzing rattles. One more rather widespread manifestation of a kartsinoidny syndrome are belly-aches which can be caused by a mechanical obstacle to the movement of intestinal contents because of growth of primary tumor or emergence of the secondary centers in an abdominal cavity.
Kartsinoidny crisis – the state arising during surgeries, which is followed by the sharp lowering of arterial pressure, increase of warm reductions expressed by a bronchospasm and increase in level of glucose in blood can become a terrible complication of a kartsinoidny syndrome. The shock which arose during kartsinoidny crisis poses a direct threat for life of the patient and can cause a lethal outcome.
Diagnostics of a kartsinoidny syndrome
The diagnosis is established by the oncologist on the basis of characteristic symptoms, the given external survey and objective researches. In plasma of blood the increased serotonin level comes to light. In urine the high content of 5-oxyindolacetum acid is defined. For an exception of false positive result within 3 days before testing to the patient with suspicion on a kartsinoidny syndrome recommend to refrain from reception of the products containing a large amount of serotonin (walnuts, eggplants, avocado, tomatoes, bananas etc.) and some medicines.
In doubtful cases for stimulation of inflows carry out tests with alcohol, catecholamines or a gluconate of calcium. For definition of localization of a tumor and identification of metastasises of the patient with a kartsinoidny syndrome direct to KT and MPT of internals, a stsintigrafiya, a X-ray analysis, gastroscopy, a kolonoskopiya, a bronkhoskopiya and other researches. In the presence of sufficient indications the laparoscopy can be used. The Kartsinoidny syndrome is differentiated with a system mastotsitoz, inflows during a climax and side effects at reception of some medicines.
Treatment of a kartsinoidny syndrome
The main method of treatment is surgical intervention. Depending on localization and prevalence of process perhaps radical excision of primary center or various palliative operations. At the kartsinoidny syndrome caused by tumors of a small intestine usually carry out a resection of a small intestine together with the site of a bryzheyka. At the damage of a large intestine and new growths located in an ileotsekalny sphincter carry out a right-hand gemikolektomiya.
At the kartsinoidny syndrome caused by a new growth in the field of an appendix carry out an appendektomiya. Some oncologists in addition delete nearby lymph nodes. Palliative surgical interventions are shown at metastasis stage, provide removal of the large centers (both secondary, and primary) for decrease in level of hormones and reduction of expressiveness of a kartsinoidny syndrome. As one more way to reduce manifestations of a kartsinoidny syndrome serves the embolization or an alloying of a hepatic artery.
The indication to carrying out chemotherapy at a kartsinoidny syndrome is existence of metastasises. Besides, this method of treatment is applied in the postoperative period at damage of heart, violations of functions of a liver and high level of 5-oxyindolacetum acid in urine. To avoid development of kartsinoidny crisis, treatment is begun with small doses of medicines, gradually increasing a dosage. Efficiency of chemotherapy at a kartsinoidny syndrome is low. Significant improvement is noted no more than at 30% of patients, the average duration of remission makes 4-7 months.
As symptomatic therapy at a kartsinoidny syndrome appoint serotonin medicines antagonists: fluoxetine, , , etc. At the tumors producing a large number of a histamine apply , ranitidine and . For elimination of diarrhea recommend to accept loperamide. Besides, at a kartsinoidny syndrome widely use and which suppress secretion of biologically active agents in the field of a new growth and allow to reduce significantly manifestations of this syndrome more than at a half of patients.
The forecast at a kartsinoidny syndrome is defined by prevalence and degree of a zlokachestvennost of a tumor. During radical removal of primary center and lack of metastasises perhaps absolute recovery. At metastasis the forecast worsens. Because of slow progressing of a kartsinoid life expectancy in such cases fluctuates from 5 to 15 and more years. Multiple metastasises in the remote bodies, exhaustion, heart failure or intestinal impassability become the reason of a lethal outcome.