It is not typical for pheochromocytoma. Pheochromocytoma - what is it? Pheochromocytoma: symptoms, diagnosis, treatment, photo. Video: an example of a laparoscopic surgery to remove a pheochromocytoma

Pheochromocytoma (PCC) is a neoplasm that appears as a result of abnormal proliferation of chromaffin cells. The tumor is hormonally active. It develops against the background of arterial hypertension. The main treatment is surgery.

What is a pheochromocytoma

Pheochromocytoma(ICD-10 code D35) is an endocrine tumor, which consists of pheochromic cells that produce excess adrenal hormones - dopamine, norepinephrine, adrenaline. These substances are called catecholamines. Hyperfunction of hormones leads to an increase in blood pressure, causes vascular disorders, changes in the myocardium and kidneys. As a rule, the disease manifests itself in the form of a hypertensive crisis.

Pathogenesis: under the influence of adrenaline, the heart rate increases, the systolic blood pressure rises. This hormone also causes vasospasm, hyperglycemia. Under its influence, lipolysis is enhanced (the metabolic process of the breakdown of fats into their constituent fatty acids under the action of lipase).

Norepinephrine increases blood pressure, causing skeletal muscle vasospasm, thereby increasing peripheral resistance. A high amount of hormones leads to strong agitation, sweating, fright, tachycardia.

Dopamine affects the psychoemotional state of a person. Also, under the influence of a large number of these hormones, disorders occur in the vegetative-vascular system.

Long-term arterial hypertension and hypercatecholaminemia can lead to changes in the myocardium and the development of catecholamine myocardial dystrophy, which eventually leads to non-coronary necrosis of the myocardium. Excessive amounts of catecholamines lead to peripheral vasospasm and the development of arterial hypertension.

What is pheochromocytoma and what do diseased organs look like in the photo:

Pheochromocytoma can be benign or malignant. The oncological process develops only in 10 cases out of 100. The malignant tumor has extra-adrenal localization and produces dopamine. Such neoplasms metastasize to regional lymph nodes, bones, liver, muscles, lungs.

A tumor develops in one of the adrenal glands (more often in the right). It can be located inside the gland or near it. An isolated tumor produces only norepinephrine. A benign neoplasm is enclosed in a capsule, which is well supplied with blood thanks to numerous blood vessels. The malignant has no membrane, it is represented by affected cells. The tumor rarely affects both adrenal glands. The size of the neoplasm is 0.5-14 cm. It increases by 3-7 mm annually. It can weigh about 70 grams.

In 90% of cases, the tumor is located in the adrenal medulla, in the aorta - in 8%. The chest and abdominal cavity accounts for only 2%. FHC is extremely rare. The disease most often affects women between the ages of 20 and 50. Sometimes pheochromocytoma occurs in children, mainly in boys.

Causes of pheochromocytoma

Pheochromocytoma of the adrenal gland secretes hormones that affect the well-being of patients. The cardiovascular, endocrine, and nervous systems suffer from hyperfunction of catecholamines.

The disease is typical for people with high blood pressure. Hypertension can be episodic or persistent. An increase in pressure can provoke emotional experiences, physical activity, overheating, alcohol intake, certain medications, deep palpation of the abdomen in the area of ​​the affected adrenal gland. The pathological process in the adrenal glands develops due to various factors.

Causes of the disease:

• hereditary - in 10 cases out of 100 the parents of the patients had adrenal cancer;
• genetic - the disease is caused by a violation of the chromosomes responsible for the production of hormones by the adrenal glands;
• due to Sipple's or Gorlin's syndrome - there are disorders in the work of the adrenal glands, thyroid gland, musculoskeletal system and in the mucous membranes.

Most often, adrenal pheochromocytoma appears unexpectedly, its occurrence may not be associated with poor heredity. The development of the disease is influenced by various chronic pathologies that reduce human immunity. Frequent stresses, infectious diseases, bad habits, living in a territory contaminated with toxic emissions and radiation can provoke disturbances in the work of the glands.

The clinical symptoms of the disease are associated with the effect on the body of an excessive amount of catecholamines, which the tumor produces. In addition to these hormones, PChC can produce somatostatin, serotonin, calcitonin and other substances that cause various disorders in the body.

Symptoms

Pheochromocytoma of the adrenal gland is sometimes characterized by a prolonged asymptomatic course. Sometimes, throughout a person's life, the disease does not make itself felt in any way. FHC has 2 forms: asymptomatic and clinically pronounced.

The latent stage of the disease is manifested by a hypertensive crisis with strong emotional or physical stress. Clinically pronounced FHC is accompanied by an increase in blood pressure, frequent pulse, decreased vision, pain in the abdomen, frequent urination, fever up to 40 degrees, weight loss. Against the background of persistent high pressure, crises arise, but sometimes they may not be.

Symptoms of pheochromocytoma in the presence of hypertension:

• throbbing headache;
• nausea;
• chills;
• excessive sweating;
• pallor;
• increased heart rate;
• feelings of discomfort in the chest.

A study for the presence of FHC should be carried out with the following signs:

• panic attacks;
• hyperventilation syndrome;
• increased consumption of coffee;
• hot flashes during menopause;
• fainting;
• poisoning;
• convulsions.

Pathologies for which differential diagnostics is performed at FHC:

• psychosis;
• neurosis;
• thyrotoxicosis;
• encephalitis;
• stroke;
• World Cup injury;
• hypertension;
• tachycardia.

Manifestations of pheochromocytoma:

• increased blood pressure;
• arrhythmia, tachycardia;
• severe fatigue, headache, fear;
• increased sweating, lacrimation, salivation;
• nausea, intestinal cramps, constipation, diarrhea;
• vasoconstriction of the skin, pallor;
• deterioration of vision;
• development ;
• changes in blood composition, leukocytosis, lymphocytosis, hyperglycemia;
• weight loss.

With FHC, the development of a hypertensive crisis is possible. The attack lasts a few minutes or a couple of hours and can suddenly end with a sharp drop in blood pressure. A crisis can occur once a month or several times a day. A severe attack can result in a stroke, myocardial infarction, catecholamine shock.

Important! Malignant pheochromocytoma causes weakness, abdominal pain. The patient has a sharp weight loss. The tumor can metastasize to other organs, affecting them, and regional lymph nodes.

Diagnostics

Before treating the disease, it is necessary to carry out laboratory and computer diagnostics of pheochromocytoma. First of all, the doctor assesses the symptoms and complaints of the patient, measures blood pressure, sends it to the ECG. The endocrinologist examines the patient and prescribes drug therapy. The disease is treated by a surgeon. Based on the results of the examination, the doctor prescribes the necessary tests. They are best done during a hypertensive crisis.

The laboratory diagnosis of pheochromocytoma is based on the determination of the amount of hormones (catecholamines) and their metabolites in the blood and urine. The end products of the breakdown of these substances are metanephrines, they are important for determining the picture of the disease. Their indicator is revealed on the basis of urine analysis.

Total metanephrine rates:

Normmetanephrine rates:

An increased level of metanephrines is noted in the urine. Analyzes say that this is FHC. The indicators of glucose and protein are also increased. Microscopic examination reveals the cylinders.

Before passing a urinalysis, the patient must prepare. You should exclude foods containing serotonin from your diet. It is necessary to stop taking all medications, avoid stress, physical activity, and give up bad habits.

Diagnosis of pheochromocytoma includes blood tests for:

• calcitonin;
• chromogranin A;
• aldosterone;
• ACTH;
• renin;
• blood cortisol.

A complete blood count can show lymphocytosis, leukocytosis, erythrocytosis, and eosinophilia. Biochemical - increased glucose levels.

During a crisis, the amount of catecholamines in the blood increases by several tens (hundreds) times. Moreover, there may be more norepinephrine than adrenaline. An increased amount of dopamine indicates a malignant process.

The results of a blood test with FHC against the background of a hypertensive attack:

• leukocytes - above 9.0 x 109 / l;
• lymphocytes - more than 37% of the total number of leukocytes;
• eosinophils - above 5% of the total number of leukocytes;
• erythrocytes - more than 5.0 1012 / l;
• glucose - above 5.55 mmol / l.

Normal blood levels of catecholamines:

• norepinephrine - 95-450 pg / ml;
• adrenaline - 10-85 pg / ml;
• dopamine - 10-100 pg / ml.

If the diagnosis of the level of catecholamines has not yielded results, functional tests can be done - provocative or adrenolytic. Stimulation tests pose a health hazard and are rarely used. Provocative tests stimulate the tumor to release catecholamines. The test is done against the background of the development of a hypertensive crisis. In the blood and urine, an increased content of hormones is detected.

Instrumental methods are used to visualize the tumor:

• radioisotope scanning;
• angiography.

With ultrasound, the size and shape of the tumor can be seen. The image shows the contents of the neoplasm (fluid, calcifications). CT and MRI provide information about the nature of the tumor. Diagnosis is carried out using contrast injection. A radioisotope scan allows you to find tumors that are outside the adrenal gland, as well as metastases. Angiography is used to draw blood from veins. With this method, the level of catecholamines is determined.

Pheochromocytoma treatment

The main method of treating the disease is surgery. Before the operation, the patient is prescribed drug therapy. Before surgery, the patient should relieve the symptoms of a hypertensive crisis.

To normalize blood pressure and stop tachycardia, appoint a- and b-blockers: Tropafen, Phentolamine, Phenoxybenzamine, Metoprolol, Propranolol... To reduce the force of heart contractions and lower pressure, use calcium channel blockers (Nifedipine)... To suppress the production of adrenaline and norepinephrine an inhibitor of catecholamine synthesis (metyrosine) is prescribed.

Method of using medicines:

• Tropafen - the drug is administered intravenously every 5 minutes until the symptom of a crisis is removed, 1 ml of a 1% solution is diluted with 10 ml of NaC solution;
• Phentolamine - taken orally at 0.05 g 3-4 r. a day after meals for 3-4 weeks;
• Propranolol - intravenously 1-2 mg every 5-10 minutes until the crisis is relieved;
• Methyrosine - 250 mg taken orally 4 r. per day, subsequently the dose is increased to 500-2000 mg;
• Nifedipine - 10 mg orally 3-4 r. in a day.

During surgery, a total adrenalectomy is performed. In this case, the affected adrenal gland is removed along with the tumor. Due to the high probability of the location of the tumor outside the gland and the presence of multiple neoplasms, the laparotomy approach is preferred. In some cases, laparoscopic intervention is allowed. If there is multiple endocrine neoplasia, both adrenal glands are resected.

Before the operation, the patient undergoes special training. He is re-prescribed blood tests, urine tests, MRI, X-ray, ECG. 5 days before surgery, the patient takes medications to normalize blood pressure and improve heart function, as well as fortifying drugs.

Preoperative course of drug treatment:

• medicines that reduce the activity of adrenaline;
• drugs that normalize blood pressure;
• medicines that decrease the heart rate;
• vasodilating drugs.

Indications for surgery:

• hormone-active FHC;
• hormonally inactive neoplasm of more than 4 cm.

Contraindications:

• poor blood clotting;
• too high or low blood pressure, not amenable to correction;
• the presence of severe chronic pathologies;
• advanced age (over 70 years).

Operation methods:

• open access- during the operation, a long oblique incision is made under the ribs, the recovery period is several weeks;
• laparoscopic access- 3 incisions of 1.5 cm are made on the abdominal wall, surgical instruments and an endoscope with video equipment are inserted through them, recovery takes 3-5 days;
• retroperitoneoscopic surgery- access to the tumor is carried out through the lower back, during the operation, the vessels are cut off, and the neoplasm is removed using a special technology.

During the operation, doctors constantly monitor hemodynamics. After removal of the tumor, blood pressure should go down. If this did not happen, then such a complication indicates damage to the renal artery or incomplete removal of the neoplasm. Blood and urine tests for catecholamines should be repeated 8 days after the operation. Arterial hypertension can also be an independent hypertensive disease. To find out the cause of high blood pressure conduct a test with Clonidine or Phentolamine.

If FHC is found in pregnant women, then they first undergo blood pressure stabilization. Then an abortion or caesarean section is performed. Then the tumor itself is removed.

If a malignant neoplasm is found in a patient, then a course of chemotherapy is prescribed after the operation. Treatment is carried out as follows cytostatic drugs: Vincristine, Dacarbazine, Cyclophosphamide.

With multiple tumors, there is no single standard for surgery. The neoplasm is resected in several stages. Due to the high risk, you have to remove the tumor in parts.

With pheochromocytoma, conservative treatment is not used. It is ineffective. The drugs only reduce the amount of catecholamines in the body, but do not affect the tumor. Medication is mainly aimed at preventing the development of a hypertensive crisis. Taking adrenergic blockers for too long can lead to mental disturbances and disturbances in the work of the gastrointestinal tract.

Forecast

After removal of a benign neoplasm, the prognosis is favorable. After the operation, normalization of blood pressure is noted, a gradual regression of pathological symptoms. The five-year survival rate in this case is 95%. In 10 cases out of 100, relapses are possible. In the postoperative period, you need to be under the supervision of an endocrinologist and regularly (at least 1 time per year) undergo examinations.

In the absence of treatment, as well as after removal of the malignant tumor, the prognosis is less favorable. In this case, the patient can live no more than 5 years. When FHC is detected in pregnant women, the mortality rate is almost 50% (for both the mother and the child).

Complications

The disease must be diagnosed in time and treated in a timely manner. Otherwise, the patient may experience various disorders in the work of the body. During treatment, you should adhere to the doctor's recommendations and avoid factors that aggravate a difficult situation.

Complications of FHC:

• myocardial infarction;
• arrhythmia;
• stroke;
• visual impairment, blindness;
• pulmonary edema.

The disease can lead to development. FHC often causes renal vascular atherosclerosis. Severe forms of the disease lead to impaired cerebral circulation and even death.

Prevention

As a prevention of the disease, it is necessary to lead a healthy lifestyle. Eat lots of fresh fruits and vegetables. Diversify your diet with seafood, lean meat, eggs, nuts, dairy products, cereals.

You should be in the fresh air every day, engage in moderate exercise. You must be able to protect yourself from the effects of stress and a variety of negative factors.

There is a risk group, which includes people most susceptible to pheochromocytoma. These are women between the ages of 30 and 50, boys under 14, people with hereditary genetic pathologies. They should be regularly examined by an endocrinologist.

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Pheochromocytoma originates from cells in the central part of the adrenal gland. It can occur at any age, including in children, but more often it is found at the age of 20-50.

The adrenal glands produce a number of biologically active substances that are vital for the body. For example, the hormones adrenaline and norepinephrine are synthesized; they are also called catecholamines or fight-or-flight hormones. The fact is that in various stressful situations, these substances are released into the blood, regulating the heart rate, metabolism and blood pressure and forcing the body to act when danger arises. Most pheochromocytomas are diagnosed in the central (cerebral) part of one or both of the adrenal glands.

Blood pressure with pheochromocytoma

The tumor produces excessive amounts of adrenaline and norepinephrine, leading to a rapid heartbeat, sudden rises, or persistently high pressures. According to statistics, pheochromocytoma is an infrequent cause of arterial hypertension (0.2-0.6% of all cases). Nevertheless, a sharp increase in blood pressure in young people requires the exclusion of this disease, since a significant rise in pressure is dangerous for the development of cardiovascular complications.

Causes of pheochromocytoma

It is still unknown what causes the development of the disease. It was found that the tumor begins its development from the chromaffin cells of the adrenal medulla. It is these cells that produce epinephrine (adrenaline) and norepinephrine (norepinephrine).

The risk of getting sick increases if the patient already has:

• multiple endocrine neoplasia type II or MEN II. Men suffer from this rare hereditary disease, tumors develop in the thyroid and parathyroid glands, on the lips, in the tongue, in the gastrointestinal tract.
• Hippel-Landau disease, when multiple tumors arise in the nervous and endocrine system, pancreas and kidneys.
• type 1 neurofibromatosis: multiple tumors appear in the skin - neurofibromas, pigmented spots appear on the skin, and a tumor of the optic nerve also occurs.
• hereditary paraganglioma / pheochromocytoma syndrome, which occurs due to mutations in genes.
• Carney's triad: paragangliomas, pulmonary chondroma, stromal tumor of the stomach.

Classification

Allocate the formation of the adrenal glands, i.e. actually pheochromocytoma. In addition to the adrenal glands, a small number of chromaffin cells in the form of small clusters can be found in the heart, head, neck, bladder, along the spine. Tumors in these cells are called paragangliomas. They have a similar effect to pheochromocytomas on the body, since the same hormones are released.

ICD-10 classification of pheochromocytoma

The disease is listed in section D35.0 - Benign neoplasm of other and unspecified endocrine glands.

Symptoms and signs of adrenal pheochromocytoma

The main signs that will help to suspect a disease are:

• high blood pressure
• increased, rapid, or irregular heartbeat
• profuse sweating for no reason
• severe headache
• pallor of the face
• shortness of breath.

More rare symptoms may include:

• anxiety, oppressive expectation of trouble
• abdominal pain, constipation
• weight loss.

Catecholamine crises

The disease is characterized by the sudden onset of symptoms. These manifestations are called catecholamine crises. Their duration is 15 to 20 minutes. An attack can occur with a frequency of several times a day or less often; in severe cases, there can be up to 25 crises per day. The attack usually ends as suddenly as it occurs. In the period between crises, blood pressure may drop to normal or remain high.

Possible triggers for crises:

• physical exercise
• stress, nervous strain
• change in body position
• intestinal peristalsis
• contractions or childbirth.

Eating foods rich in tyramine can also trigger a crisis. This biogenic amine is abundant in fermented foods, for example:

• in mature cheeses
• in dried and smoked meat
• in avocado, bananas, beans
• in pickled fish
• in sauerkraut
• in some beers.

The release of hormones can be triggered by drugs (amphetamine, cocaine) and some drugs:

• vasoconstrictor drugs (decongestants)
• monoamine oxidase inhibitors
• dopamine receptor blockers
• serotonin reuptake inhibitors
• muscle relaxants
• glucocorticosteroids.

Diagnostics of the pheochromocytoma

The endocrinologist begins the diagnosis with a questioning, finding out the hereditary predisposition, studying the history of the disease. Then he measures the blood pressure level, counts the heart rate.

Analyzes

• determination of metanephrine and normetanephrine in urine collected per day
• determination of the level of free metanephrine in blood plasma.

Currently, the measurement of urine catecholamines and vanilla-mandelic acid levels is not used, since false-positive results are frequent.

At elevated levels of biologically active substances, imaging methods are used in the analyzes to help identify a tumor:

• computed tomography (CT) of the abdominal organs, adrenal glands. The method allows you to identify formations larger than 5 mm.
• scintigraphy, which is used if there is a suspicion of a tumor outside the adrenal glands. When conducting a study, it is necessary to take into account the possibility of asymmetric accumulation of the isotope in normal adrenal glands.
• positron emission tomography (PET). This method is more sensitive than scintigraphy in terms of detecting metastatic lesions.

If several family members have the disease or develop before the age of 40, then genetic testing is performed. Genetic testing is rarely prescribed for patients over 50 years of age.

Pheochromocytoma treatment

In rare cases, taking medications can help control blood pressure. Removal of the tumor is considered the main method of treatment.

Surgical treatment of pheochromocytoma

The operation to remove the adrenal gland - adrenalectomy is performed either laparoscopically, with the introduction of miniature surgical instruments through small incisions on the abdomen, or using open access to the tumor, when an incision is made on the abdominal skin. With a large lesion (more than 8 cm), an open method is preferable, since it is important not to damage the tumor capsule during removal. Both types of operations are performed under general anesthesia.

If malignancy of pheochromocytoma is detected, then the treatment options are as follows:

• surgical removal of the tumor
• radiation therapy
• chemotherapy
• ablation of the tumor using radio waves or cryotherapy
• embolization therapy by blocking the artery feeding the tumor
• targeted therapy by blocking certain enzymes, proteins or molecules that affect tumor growth.

Medicines prescribed before surgery

To prepare for the operation 7-14 days before it is prescribed:

• alpha blockers. These drugs reduce the effect of excess norepinephrine on the body and stabilize blood pressure by relaxing the vascular muscles. Doxazosin, prazosin, phenoxybenzamine are commonly used.
• beta blockers, which also lower blood pressure and heart rate by blocking the action of adrenaline. Used, for example, atenolol, metoprolol and propranolol.
• calcium channel blockers such as diltiazem, amlodipine, nifidepine. Since diltiazem gently reduces blood pressure and acts for a relatively short time, it is used with a slight increase in blood pressure.

Often, calcium channel blockers are added to alpha and beta blockers.

Emergency care for a sharp increase in blood pressure

If a patient with pheochromocytoma has a sharp increase in pressure, then an urgent need to call a doctor and, under his supervision, reduce the pressure. The following are used as emergency drugs:

• phentolamine 5 to 20 mg IV
• tropafen 1%, 1-2 ml IV, very slowly
• sodium nitroprusside, starting at 0.25 mcg per kilogram of body weight per minute intravenously.
• labetalol 100 mg orally. It is possible to administer intravenously at a dose of 20 mg, i.e. 1% solution is injected in an amount of 2 ml.

The use of methyldopa, minoxidil, pentamine, benzohexonium is undesirable.

Complications of pheochromocytoma

The constant production of hormones increases blood pressure and can damage the heart, kidneys, and brain. Life-threatening conditions are possible, such as:

• arrhythmia
• myocardial infarction
• stroke
• renal failure
• acute respiratory distress syndrome.

Catecholamine shock

This complication is due to the fact that catecholamines are not inactivated and continue to act on the vessels. In this case, the pressure change becomes unpredictable, episodes of pressure increase in a chaotic manner are replaced by hypotension. For treatment, alpha-blockers are used, in severe cases, hospitalization in the intensive care unit is necessary.

Malignant pheochromocytoma

Rarely, malignancy may occur, i.e. malignancy of education. This condition is called malignant pheochromocytoma. Confirm the diagnosis by biopsy, detection of metastases in bones or other organs.

Patient life prognosis

The chances of recovery depend on many parameters:

• benign or malignant tumor
• pheochromocytoma is located in one place or hormone-active cells are dispersed in several areas
• are there signs that indicate organ damage due to excess catecholamines
• were there any complications against the background of high blood pressure
• whether there is a recurrence (return) of the tumor after surgical treatment.

Pheochromocytoma of the adrenal gland is a name that is not known to every person. diagnosis, symptoms and treatment of this tumor are extremely important information, because sometimes neglected cases can cost their owners health and even life. However, in the case when it is found on time and treatment is started, the doctor in most cases tells his patient "live" and successfully copes with the misfortune. Treatment of pheochromocytoma is a very important and complex matter.

There is a disease that can affect the adrenal glands - pheochromocytoma. This tumor is among those that can produce hormones. However, it is set apart, because it may well cause fatal complications due to the fact that during its existence, the internal organs of a person suffer to a large extent.

This tumor of the adrenal glands is formed in chromaffin cells, which are found in the most significant amount in the medulla of the organ. However, the same tissue is found in other places of the human body: near the aorta, at the gates of the liver and kidneys, in the heart, solar plexus and other places. This tissue produces the hormones dopamine, adrenaline and norepinephrine, which are very important for normal life. Many metabolic processes cannot be carried out if this secretion is not in the blood. In addition, these hormones provide support in stressful situations and prevent surges in blood pressure.

In the event that a person is healthy, the adrenal glands produce the listed substances on an ongoing basis. However, their amount in the blood of the body has to be increased if an unfavorable time has come, for example, an injury has been received or stress has come. This increase affects people as follows:

• adrenaline leads to increased pressure, the heart begins to contract more and more often, more sugar appears in the blood, and the gastrointestinal tract and skin capillaries constrict the spasm.
• Norepinephrine acts very similarly, but along the way it also causes many emotional manifestations: a person feels a sense of fear and excitement, becomes covered with sweat and sweats profusely.
• The increase in dopamine levels is reflected in the vegetative state and psychoemotional area.

This situation is observed normally when a person is not sick with pheochromocytoma. With pheochromocytoma, adrenal tissue produces hormones without coordinating their amount with the situation in which a person is now. Under the constant influence of an increased amount of substances, a person is, as it were, in a constant state of stress, which eats away his body as a whole and some organs, such as the heart and kidneys, in particular.

If a person has a pheochromocytoma, diagnosis cannot be late. Only in case of rapid detection, it is possible to urgently perform an operation, removing all foci of neoplasm. And even in this case, it is not always possible to eliminate manifestations of a disease such as hypertension.

Causes of pheochromocytoma

If the question of what is pheochromocytoma does not cause difficulties for specialists, then why this diagnosis appears, in many cases it is not possible to understand. About every tenth case is the result of heredity. This, one might say, is somewhat successful, because the disease is transmitted according to a dominant characteristic, and most likely, many close relatives already have the same attack. The success of such a reason is that a person knows about the possibility of such a disease appearing in him and can be more attentive to himself so as not to miss the moment when it is necessary to start treatment.

Examination reveals a mass surrounded by a capsule. Most often, its dimensions fluctuate within five centimeters. Moreover, its size is not at all important for the ability to produce hormones. Moreover, this adrenal tumor produces not only the three hormones already mentioned above, but also some others, such as serotonin or calcitonin.

Most often, education settles only in one of the paired organs, but there are also cases of paired lesions. Diagnosis of a pheochromocytoma usually shows what the nature of the tumor is. In the event that it belongs to malignant, it is called pheochromoblastoma. In this case, chromaffinoma can go beyond the main affected organ and grow with metastases in the lungs, bones, lymph nodes and liver. The prognosis of how good a person's chances of survival and recovery are, depends on the number of metastases.

Pheochromocytoma symptoms

If a person develops a pheochromocytoma, its symptoms will not be long in coming. The thing is that this tumor stands out from the group of its own kind by the strongest suppression of many vital functions of the body. Depending on the manifestations of the disease, doctors divide it into three forms: paroxysmal, permanent and mixed.

Paroxysmal form of the disease

Studies show that 85 out of a hundred patients suffer from paroxysmal form. In this case, the patient experiences typical signs of a hypertensive crisis:

• The head hurts very badly. Sometimes the pain is accompanied by dizziness.
• A person cannot move normally, since any effort is accompanied by shortness of breath.
• Symptoms also include pain in the region of the heart.
• The person shows anxiety, he is pale and subject to an inexplicable feeling of fear.
• With this diagnosis, sweating increases, the number of heart contractions increases.
• Profuse urination occurs.
• Nausea rolls up, convulsive syndrome manifests itself. Also, a person is in a fever and is tormented by thirst.

Many of the above symptoms may not manifest themselves at the same time, however, there are three signs that almost 100% of patients complain about: headache, increased sweating and rapid pulse.

As the name implies, symptoms of this type of pheochromocytoma are not constantly present. An attack appears suddenly and anything can act as a provocateur: a person can overdo it with physical exertion, eat junk food, drink an alcoholic drink, become overly anxious, or even just unsuccessfully urinate. A sharp jump in pressure is likely to await someone who is trying to grope a tumor of the adrenal glands.

The attack, if you're lucky, can release in a couple of minutes, but in difficult cases, there are often crises that last several hours. It is very typical for a disease to have an unexpected end to an attack. In this case, the patient can discharge up to five liters of urine, and he himself will be literally wet with sweat. The patient is overcome with weakness and a feeling of incredible fatigue.

Persistent pheochromocytoma

Diagnosis of pheochromocytoma should be carried out at an accelerated pace when therapy is required for a persistent type of disease. With such hypercatecholaminemia, blood pressure is high on an ongoing basis and does not decrease after an exacerbation, as in the previous case. This condition is similar to that of a person diagnosed with primary hypertension. A patient with this form demonstrates the following signs of pheochromocytoma:

• A person is constantly in a state of irresistible weakness.
• This adrenal swelling causes people to be emotionally unstable.
• It is not known what concomitant diseases can join this education. Disorders of metabolic processes, for example, carbohydrate, are quite possible. This means that the blood glucose may increase and a person will have to build a life prognosis not only for adrenal tumors, but also for diabetes mellitus.

Mixed pheochromocytoma

Treatment of mixed pheochromocytoma is a very urgent matter. Indeed, in this case, the study will show not only a constant increase in blood pressure or its temporary jumps. The therapeutic effect requires both that, and another together. Moreover, a crisis can still arise literally out of nothing, stress can be considered the most tangible shocks, and other reasons cannot even be predicted.

Every tenth adult patient (in children, this occurs in a greater number of cases) demonstrates an acute pathogenesis, which is called catecholamine shock. This dangerous condition is described as a sudden change in the patient's high blood pressure to low. No one knows how many such jumps in a row the body can withstand, so when a pheochromocytoma shows itself in this way, treatment should be done especially quickly.

Any of the crises that come on can end up with severe and sometimes fatal complications for the patient. These can be severe kidney damage (failure), pulmonary edema, and even a heart attack. Therefore, in the case when a patient is diagnosed with pheochromocytoma, it is extremely difficult for a doctor to make a prognosis.

Asymptomatic course

Sometimes a person develops such a pheochromocytoma, the symptoms and diagnosis of which defy any description. A tumor can "disguise" itself as many other possible human diseases, such as a heart attack, acute diseases of the gastrointestinal tract, various kidney diseases, or even a stroke. And especially "cunning" neoplasms can hide at all and do not show their presence in any way. In this case, the doctor cannot immediately understand that the patient has a pheochromocytoma, and he may not take the tests that are needed.

How many such tumors are without signs, statistics cannot answer. Indeed, in this case, people do not go to doctors, because they are not worried about anything. The tumor can grow to the size of several kilograms, and show its presence with some slight tingling in the back. In this case, treatment of pheochromocytoma does not come and death can occur suddenly, for example, due to the fact that hemorrhage occurs in the tumor itself. Any asymptomatic attack from stress or excessive exertion can provoke this condition. In this case, only the pathologist at the autopsy will find out the reason for it.

Pheochromocytoma (chromaffinoma) is a tumor of chromaffin tissue that secretes catecholamines (adrenaline, norepinephrine, dopamine) and is localized in the adrenal medulla or sympathetic paraganglia. Depending on the localization, pheochromocytomas are divided into adrenal and extra-adrenal - paragangliomas.

Pheochromocytomas are found in 0.1% of patients with arterial hypertension. They are the cause of symptomatic hypertension, which disappears when the tumor is removed. And at the same time, pheochromocytoma can be fatal with the development of a severe sympathoadrenal crisis with high uncontrollable blood pressure, a high risk of myocardial infarction or cerebral stroke.

Etiology and pathogenesis

The causes of the disease have not been definitively established. It is known that pheochromocytomas develop in two adrenal glands simultaneously in 10% of patients, in 10% of cases the tumor is located outside the adrenal glands and about 10% of them have signs of malignancy. Solitary pheochromocytomas often develop mainly on the right and have a diameter of less than 10 cm.

About 5-10% of cases of pheochromocytoma is a familial pathology and is inherited as an autosomal dominant feature either independently or as part of another pathology (multiple endocrine neoplasia), combined with medullary thyroid cancer, neurofibromatosis, and retinal cerebellar hemagioblastomatosis. In familial syndromes, bilateral adrenal pheochromocytomas are more common. The genetic basis for the inheritance of pheochromocytoma is provided by point mutations and a RET-proto-oncogene in the long arm of the 10th autosome, encoding the structure of the tyrosine kinase receptor.

Extra-adrenal pheochromocytomas, as a rule, are significantly smaller (diameter less than 5 cm), and most of them are located in the paravertebral space of the abdominal cavity, about 1% of pheochromocytomas are localized in the chest cavity, 1% in the bladder and less than 1% in the area neck. Possible localization of pheochromocytoma in the pericardium, in the brain.

Pheochromocytomas can have signs of malignancy (pheochromoblastoma), which are difficult to identify from the histological picture. Malignancy is determined by local invasion of the surrounding tissue or distant metastases. Pheochromoblastomas metastasize to the liver, bones, lungs, and lymph nodes.

The pathogenesis of pheochromocytoma is realized through the effect of an excess of catecholamines on organs and systems, among which the cardiovascular and nervous systems occupy the main place. In addition, the qualitative composition of the secreted catecholamines is important. The mechanism of release of catecholamines from pheochromocytomas is the result of changes in blood flow in tumor tissue or necrosis of tumor tissue. Since pheochromocytomas are not innervated, the secretion of catecholamines does not occur as a result of nerve stimulation.

Most pheochromocytomas secrete norepinephrine and, to a lesser extent, epinephrine. Most extra-adrenal pheochromocytomas exclusively secrete norepinephrine. Isolated production of adrenaline by pheochromocytomas is rare, mainly in MEN. The production of dopamine and homovanillic acid (HVA) is significantly increased, mainly in malignant tumors.

Symptoms

Pheochromocytoma is more common in middle age. A characteristic feature of pheochromocytomas is the polymorphism of clinical manifestations, however, most patients go to the doctor for an increase in blood pressure, which is difficult to respond to conventional treatment, or paroxysms of vegetative symptoms, accompanied by increases in blood pressure.

Most patients develop sympathoadrenal crises against the background of persistent hypertension. In a minority of patients, blood pressure rises only during an attack. Blood pressure often reaches high values, hypertension is sometimes malignant and resistant to treatment with standard antihypertensive drugs. Crises are observed in more than half of patients with pheochromocytoma and can be both sporadic, with long intervals (up to several weeks or even months), and frequent. Over time, the frequency, duration and severity of crises tend to increase.

In most cases, the crisis starts suddenly and can last from a few minutes to several hours. At the same time, an attack can be triggered by any activity that causes displacement of the abdominal organs (physical work, palpation of the abdominal organs) or hypothermia. Stress or psychological stress usually does not trigger the development of a crisis.

The crisis is characterized by the appearance of a headache, profuse sweating, palpitations, a rapid rise in blood pressure to threatening values ​​(> 250/130 mm Hg), acute pain in the chest and abdomen with nausea and vomiting. Consciousness during the development of the crisis is preserved, the patients are agitated and note a feeling of fear and fear of death. During an attack, the complexion may change (pallor or hyperemia), pupils dilate, severe tachycardia, profuse sweating are noted. Development of epileptiform seizures is possible. After a crisis, it is noted with the discharge of a large amount of urine with a low relative density. Crises are characterized by a paradoxical reaction (increased blood pressure) to standard antihypertensive therapy, may result in retinal hemorrhage, myocardial infarction, cerebral stroke, pulmonary or cerebral edema, and a legal outcome.

In addition to the development of crises, with a prolonged course of pheochromocytoma, symptoms of metabolic activation are noted - the weight significantly decreases, hyperthermia is possible.

Cardiac manifestations of pheochromocytoma are characterized by rhythm disturbances (sinus tachycardia or sinus bradycardia, ventricular premature beats). The appearance of angina pectoris or the development of myocardial infarction is possible even in the absence of significant damage to the coronary arteries. The cause of myocardial ischemia is considered to be coronary spasm and an increase in oxygen consumption by the myocardium against the background of an excess of catecholamines.

On the electrocardiogram, nonspecific changes in the ST segment and T wave, pronounced U-waves, signs of left ventricular tension and blockade of the right and left bundle branch in the absence of obvious ischemia or infarction can be detected. With a long course of arterial hypertension, cardiomyopathy develops (hypertrophic with concentric or asymmetric myocardial hypertrophy), which may be accompanied by heart failure and cardiac arrhythmias.

A symptom characteristic of pheochromocytoma is orthostatic hypotension with possible collapse (due to a decrease in plasma volume and a slowdown in sympathetic reflexes).

About 50% of patients, due to inhibition of insulin secretion and stimulation of glucose production by the liver, have disorders of carbohydrate metabolism (impaired glucose tolerance or secondary), which disappear after removal of the pheochromocytoma. In addition, erythrocytosis is noted (due to the activation of production, erythropoietin) and / or an increase in hematocrit (due to a decrease in plasma volume).

One of the relatively common localizations of extraadrenal pheochromocytoma is its location in the wall of the bladder. Clinically, pheochromocytomas of the bladder can manifest with typical attacks (crises) during urination and hematuria.

Some drugs (opiates, histamine, ACTH, glucagon) can provoke the development of severe crises, which is due to the direct stimulation of the release of catecholamines from the tumor. The development of a crisis can also be stimulated by methyldopa, which increases blood pressure due to the release of catecholamines from increased reserves in neurons, as well as substances that block the neuronal uptake of catecholamines (tricyclic antidepressants, guanethidine) and enhance the physiological effects of endogenous catecholamines.

As mentioned above, pheochromocytoma can be a component of familial syndromes of multiple endocrine neoplasia and be combined with medullary thyroid cancer, neurofibromatosis, etc.

In some cases, pheochromocytoma can be accompanied by manifestations with excessive production of cortisol and changes in appearance characteristic of hypercortisolism. Hypercortisolism in such cases is due to ectopic secretion of ACTH by pheochromocytoma, or, even more rarely, concomitant medullary thyroid cancer. An increase in aldosterone production is possible, not accompanied by an increased level of renin.

• acute development and possible spontaneous relief of the crisis;
• high blood pressure (> 250/130 mm Hg);
• tachycardia, cardiac rhythm disturbances, tremors, pallor of the skin and dilated pupils;
• profuse sweating and hyperthermia;
• hyperglycemia, glucosuria;
• lack of an adequate response to antihypertensive therapy.

According to the severity of clinical symptoms, 3 stages of the development of the disease are distinguished:

• Stage 1 - initial (rare attacks with short crises);
• Stage 2 - compensated (prolonged attacks lasting up to 30-40 minutes no more than 1 time per week with an increase in blood pressure up to 250 mm Hg with preservation of arterial hypertension in the interictal period;
• Stage 3 - decompensated (frequent, almost daily attacks with a high rise in treatment-resistant blood pressure).

Diagnostics

Laboratory data. The diagnosis of pheochromocytoma is confirmed by the detection in daily urine of an increased content of free (unconjugated) catecholamines or their metabolites (vanillyl mandelic acid (VMC) and metanephrins). All indicators are equal in their specificity and sensitivity, therefore, one indicator is sufficient for the diagnosis. Certain conditions must be met when collecting urine. Collect urine under resting conditions. Patients who have recently undergone a study using radiopaque contrast media are excluded. Do not prescribe drugs (sympathomimetic amines, methyldofu, levodofu) that increase the excretion of catecholamines for 2 weeks. In patients with crises, the collection of daily urine should be started from the moment of an attack of hypertension.

The urinary excretion of free catecholamines in 24 hours in patients with pheochromocytoma exceeds 250 μg (the norm is 100-150 μg / day). It is advisable to study the excretion of adrenaline, since in adrenal pheochromocytomas, the excretion of this hormone predominates. In patients, the excretion of adrenaline exceeds 50 mcg / day (the norm is 0-13 mcg / day).

The daily excretion of metanephrine in healthy people is 0-1.0 mg and IUD - 2-10 mg. In patients with pheochromocytoma, the excretion of these metabolites increases 2-3 times.

Plasma catecholamines are rarely studied at present, only with the clinical picture of pheochromocytoma and inconclusive data from the study of daily urine. Basal concentration of catecholamines is usually examined under standard conditions. It is recommended that a blood sample for catecholamines be taken using the supplied venous catheter after 30 minutes of the patient's stay in the supine position in a relaxed state. The diagnosis of pheochromocytoma is confirmed with a significant increase in plasma basal levels of norepinephrine (norm 0.09-1.8 nmol / l) and adrenaline (norm 135-270 nmol / l). But in a third of patients, they may be normal. It is advisable to conduct a test with clonidine, which reduces the tone of the sympathetic nervous system. Clonidine is prescribed in the morning on an empty stomach 0.20 mg to patients aged 35-50 years and 0.25 mg - over the age of 50 years. Immediately before and 60 and 90 minutes after taking clonidine, blood is taken to determine the level of catecholamines. In healthy and hypertensive patients, clonidine reduces the level of catecholamines in the plasma and does not affect the level of catecholamines in pheochromocytoma.

Suppressive and provocative tests. Of the suppressive tests, only the phentolamine test is used during a hypertensive crisis for the purpose of diagnosis and trial treatment. After a trial dose of 0.5 mg, phentolamine is injected intravenously at a dose of 5 mg. With pheochromocytoma, systolic blood pressure decreases by at least 35 mm Hg. Art., diastolic - by 25 mm Hg. Art. after 2 min. The antihypertensive effect lasts 10 minutes. Provocative tests are dangerous and have been almost completely discontinued.

Instrumental research. Adrenal pheochromocytomas can be detected by CT or MRI of the adrenal glands. Pheochromocytomas localized in the chest are detected by X-ray and CT, localized in the abdominal cavity by MRI. To identify a pheochromocytoma located in the abdominal cavity and not detected by MRI, aortography is performed. In addition to these methods, scintigraphy with meta-131I-benzylguanidine is used.

Differential diagnosis carried out with hypertension, catecholamine crises that develop with the withdrawal of clonidine and treatment with MAO inhibitors, and provocative crises in patients with impaired central nervous system function, taking sympathomimetics. Carrying out a study of the excretion of catecholamines in daily urine allows you to exclude these conditions.

Treatment

Surgical treatment is a radical method. Preoperative preparation is aimed at stable blockade of α-adrenergic receptors. For this purpose, phenoxybenzamine is used, which is administered orally at an initial dose of 10 mg 2 times a day. The dose is increased by 10-20 mg in a few days until the blood pressure normalizes and the seizures stop. The optimal dose for most patients is 40-80 mg per day. With the development of severe hypertensive crises, IV phentolamine is used. In some patients, long-term α-adrenergic blockade can be achieved with prazosin, a selective α1-receptor antagonist. The drug is prescribed at a dose of 1.5-2.5 mg every 6 hours.

Β-adrenergic receptor blockers should not be used in isolation, as they can cause a paradoxical increase in blood pressure. β-blockers are prescribed only after α-blockade is achieved, accompanied by tachycardia and arrhythmias. Small doses of propranolol 10 mg are prescribed 3-4 times a day. Preoperative preparation is carried out for 1-14 days.

During surgery, blood pressure, ECG and central venous pressure are continuously recorded. Complications of the operation - significant fluctuations in blood pressure, heart rhythm disturbances and shock caused by the sudden removal of the source of hyperproduction of catecholamines. Hypotension is usually controlled by fluid volume replacement, hypertension by IV phentolamine, but sodium nitroprusside may be required. Rhythm disturbances and tachycardia are controlled by intravenous administration of propranolol.

For inoperable tumors, long-term treatment with α- and β-blockers is carried out. In case of intolerance to adrenergic blockers or no effect, metirzine is prescribed - a tyrosine hydroxylase inhibitor, which blocks the formation of catecholamines by the tumor. Methirzine permanently reduces the severity of symptoms of pheochromocytoma. Irradiation and chemotherapy for pheochromocytoma are usually not used.

Forecast

After complete removal of pheochromocytoma, blood pressure normalizes in 75% of patients, in 25% of patients the pressure remains elevated, which is due either to concomitant hypertension or severe vascular hardening resulting from previous prolonged hypertension. The postoperative survival rate within 5 years reaches 95%, the recurrence rate is less than 10%.

Pheochromocytoma (chromaffinoma) is one that is formed by pathological proliferation of chromaffin cells and is characterized by hormonal activity. It most often comes from the adrenal medulla and can be either benign or malignant.

Pheochromocytomas often develop in young and mature people (20-40 years), and the prevalence among men and women is approximately the same. In childhood, the incidence is higher among boys. The tumor produces an excessive amount of peptides and biogenic amines (dopamine, adrenaline and norepinephrine), resulting in catecholamine crises.

The share of malignant types of tumors (pheochromoblastomas) accounts for less than 10% of the total number of diagnosed pheochromocytomas. For such tumors, the location outside the adrenal glands is very characteristic. Secondary foci (metastases) form in regional lymph nodes, liver, distant organs (lungs) and tissues (muscle and bone).

Etiology

As a rule, the true reason for the formation of chromaffin remains unclear.

Every tenth patient has a genetic predisposition... During the collection of the anamnesis, it turns out that these tumors were previously diagnosed in the parents. Geneticists believe that pathology is inherited in an autosomal dominant manner.

Often, pheochromocytoma is one of the manifestations of a hereditary disease - multiple endocrine neoplasia syndrome. It also affects other organs of the endocrine system - the thyroid and parathyroid glands.

Pathogenesis

Pheochromocytoma can occur not only from the adrenal medulla, but also from the aortic lumbar paraganglia (in such cases, they speak of paragangliomas). Neoplasms of this type are also detected in the pelvic area, chest and abdominal cavity. In rare cases, chromaffinoma is found in the head and neck area. Cases of tumor localization in the pericardium and heart muscle have been described.

Active substances that can be synthesized by chromaffinoma:

Pheochromocytoma is capable of producing neuropeptide Y, which is characterized by pronounced vasoconstrictor properties.

The level of hormonal activity does not depend on the size of the neoplasm (they are variable and can reach 5 cm). The average mass of a pheochromocytoma is 70 g. Rich vascularization is characteristic of these encapsulated tumors.

Adrenal pheochromocytoma symptoms

Symptoms are determined by an excess of a substance... Hypersecretion of catecholamines by pheochromocytoma leads to development. Tumors are detected in about every hundredth patient with constantly high diastolic ("lower") pressure.

The course of hypertension can be stable or paroxysmal. Periodic crises are accompanied by disorders of the cardiovascular and nervous systems, as well as digestive and metabolic disorders.

During a crisis, blood pressure rises sharply, and in the intervals between paroxysms it is stably high or returns to normal values.

Symptoms of a crisis with pheochromocytoma:

• up to 200 mm. rt. Art. and more;
• unmotivated feeling of anxiety and fear;
• intense;
• blanching of the skin;
• (increased sweating);
• cardialgia ();
• heart palpitations;

During an attack, increased leukocytosis is also noted in the peripheral blood.

The duration of the paroxysm ranges from several minutes to an hour or more. Their frequency varies from single seizures for several months to 10-15 per day. A crisis is characterized by a sharp spontaneous relief, which is accompanied by a sharp drop in blood pressure. The patient has pouring sweat and increased urine output (up to 5 liters) with a low specific gravity. He complains of general weakness and a feeling of "weakness" throughout his body.

Factors that can provoke a crisis:

• general overheating or hypothermia of the body;
• significant physical activity;
• psycho-emotional;
• taking some pharmacological agents;
• alcohol consumption;
• sharp movements;
• medical manipulations (deep palpation of the abdomen).

The most severe seizure outcome is catecholamine shock. It is characterized by uncontrolled hemodynamics - episodes and hypertension are randomly replaced and do not lend themselves to drug correction. In severe hypertensive crisis caused by chromaffinoma, complications such as exfoliation, the development of a functional one, are not excluded. Retinal hemorrhages are common. Paroxysms are of great danger for women during pregnancy.

With a stable course, the patient has a consistently high pressure, against the background of which pathologies of the heart muscle and kidneys develop over time, as well as changes in the fundus. Patients with pheochromocytoma are characterized by mental lability (mood swings and high psycho-emotional excitability), periodic cephalalgia and increased physical and mental fatigue.

Among metabolic disorders in particular is an increase in blood glucose levels (hyperglycemia), which often becomes the cause of development.

Important:with pheochromoblastomas (malignant chromaffinomas), the patient has (a sharp decrease in body weight) and pains in the abdominal region appear.

Diagnostics of the pheochromocytoma

In the course of a general examination, patients reveal a rapid heartbeat, pallor of the skin of the face, neck and chest, and increased blood pressure. Orthostatic hypotension is also characteristic (when a person gets up, the pressure drops sharply).

Important:palpation (palpation) of a neoplasm can provoke catecholamine paroxysm.

One of the important diagnostic criteria is an increase in the content of catecholamines in the urine and blood of the subject. The serum also determines the level of chromogranin-A (universal transport protein), adrenocorticotropic hormone, calcitonin and trace elements - calcium and phosphorus.

Nonspecific changes on, as a rule, are determined only during a crisis.

With pheochromacytomas, there are often concomitant pathologies - disorders of arterial circulation in the extremities (Raynaud's syndrome) and hypercortisolism with the development of the syndrome.

In a significant part of the surveyed, hypertension-related damage to the retinal vessels (retinopathy) is found. All patients with suspected pheochromocytoma need to undergo additional examination by an ophthalmologist.

In the course of differential diagnosis with chromaffin, provocative (stimulating) and suppressive tests with histamine and tropafen are used, but there is a possibility of obtaining false positive and false negative test results.

Of the hardware diagnostic methods, the most informative are ultrasound scanning and tomographic examination (s) of the adrenal glands. They allow you to clarify the size and location of the neoplasm. Additionally, they resort to selective arteriography and adrenal glands, as well as the chest organs (to confirm or exclude the intrathoracic localization of chromaffinoma).

Pathologies with which differential diagnosis is carried out:

• paroxysmal;
• some types.

Note: in pregnant women, the symptoms of pheochromocytoma are disguised as late toxicosis (gestosis) and the most severe forms of their course - eclampsia and preeclampsia.

It is far from always possible to reliably establish tumor malignancy at the preoperative stage. It is possible to speak with confidence about pheochromoblastoma if there are such obvious signs as invasion (germination) into nearby structures or distant secondary foci.

Treatment and prognosis

When pheochromocytoma is detected, drug therapy is carried out, the purpose of which is to reduce the severity of clinical symptoms and stop paroxysmal seizures... Conservative measures involve the appointment of drugs from the group α-blockers(Phentolamine, Phenoxybenzamine, Tropafen, and in the preoperative period - Doxazosin) and β-blockers(Metoprolol, Propranolol). In case of crises, it is additionally introduced Sodium nitroprusside... A very effective drug for lowering catecholamine levels is A-methyltyrosine, but its regular intake can provoke mental disorders and disorders of the digestive function.

Then surgery is performed - total adrenalectomy. During the operation, the affected adrenal gland is removed along with the tumor. Since there is a high likelihood of the location of the pheochromocytoma outside the adrenal gland and the presence of multiple neoplasms, preference is given to the "classical" laparotomy approach, but perhaps less traumatic.

Multiple endocrine neoplasia is an indication for resection of both adrenal glands.

When a pheochromocytoma is diagnosed in a pregnant patient, depending on the period, an artificial interruption is performed or, and then, the tumor is removed.

When establishing the malignant nature of the neoplasm and detecting distant secondary foci, it is indicated. Such patients need a course of treatment with cytostatic drugs - Dacarbazine, Vincristine or Cyclophosphamide.

In most cases, after resection of a benign neoplasm, blood pressure indicators normalize, and other clinical signs regress. If hypertension persists, there is reason to suspect the presence of ectopic tumor tissue, incomplete removal or accidental damage to the renal artery.

There is no single standard for surgical interventions for multiple tumors. Sometimes it is considered appropriate to carry out the resection in several stages.

The 5-year survival rate after surgery for benign chromaffinoma is 95%. After resection of pheochromoblastoma, the prognosis is less favorable.

These hormonally active tumors tend to recur in about 12% of cases. All patients who have undergone surgery are strongly recommended to be examined annually by an endocrinologist.

Plisov Vladimir, medical columnist