home Diseases Hormonally active adrenal tumor, or pheochromocytoma - clinical manifestations and diagnosis of the disease. Pheochromocytoma: symptoms, diagnosis and treatment Symptoms of pheochromocytoma

Hormonally active adrenal tumor, or pheochromocytoma - clinical manifestations and diagnosis of the disease. Pheochromocytoma: symptoms, diagnosis and treatment Symptoms of pheochromocytoma

Each adrenal gland is a small endocrine gland located in its own capsule in the retroperitoneum. The total mass of these organs is 16-24 grams. The structure of the adrenal glands is divided into medulla and cortical layers. The first of them is formed by chromaffin tissue. In origin, the cells of the medulla are close to nerve fibers. The chromaffin tissue of the adrenal glands synthesizes catecholamines (adrenaline and norepinephrine) and dopamine.

These hormones have a significant effect on the tone of the autonomic nervous system, heart rhythm, blood pressure, and metabolism. At the same time, catecholamines are not absolutely vital. If the function of the adrenal medulla decreases sharply, then it is not replaced with drugs.

Increased synthesis of hormones in chromaffin tissue is very dangerous for life and health. In most cases, such hypersecretion is provoked by a benign or malignant tumor of the adrenal glands - pheochromocytoma.

How common is the disease?

Pheochromocytoma is a fairly rare neoplasm. Every year, the disease is diagnosed in only one person per 1.5-2 million population. Among children, boys are more likely to get sick. The peak incidence occurs in young and middle age. Men are susceptible to pathology somewhat less frequently than women.

Pheochromocytoma is classified as a tumor of the APUD system. It is often detected not independently, but as part of the syndrome of multiple endocrine neoplasia. Familial forms account for about 10% of all tumors.

Malignant pheochromocytomas metastasize relatively late. An oncological process is detected in every tenth case.

Pheochromocytoma can be located outside the adrenal glands. It is sometimes found in the abdominal or chest cavity, in the neck, head, etc. Atypically localized neoplasms synthesize only norepinephrine. Their symptoms are weaker.

What disorders are caused by pheochromocytoma?

Pheochromocytoma has hormonal activity. The tumor releases inadequate doses of adrenaline and norepinephrine into the blood. Normally, these hormones are released in response to emotional stress and physical overload. The disease provokes an increase in the level of catecholamines even at rest.

Norepinephrine and adrenaline activate the heart and respiratory system, increase vascular tone, and stimulate the formation of glucose in the liver. In critical situations, such changes help a person overcome various difficulties, show aggression, defend himself, and run away. If a person is in a calm environment, then excessive secretion of catecholamines disrupts his well-being and gradually worsens his mental state and general health.

Signs of an adrenal tumor are the body’s reaction to surges in the concentration of adrenaline and norepinephrine in the blood. The higher the concentration of hormones, the brighter the clinical picture of the disease.

Hypertension in pheochromocytoma

The disease has a fairly characteristic clinical picture. But doctors sometimes do not immediately recognize the symptoms of pheochromocytoma. This is a rare pathology, so many specialists do not have sufficient practical experience in diagnosing it.

One of the obligatory signs of the disease is arterial hypertension. Hypertension is an increase in blood pressure above 140/90 mm Hg. Pheochromocytoma is often accompanied by very severe arterial hypertension. Systolic and diastolic pressure readings can even exceed 200/120 mmHg.

Hypertension with pheochromocytoma is always very persistent. Even several medications in large doses cannot stabilize blood pressure.

Hypertension can have three forms:

crisis course (sharp increases in indicators against the background of normal pressure);
crises due to constant hypertension;
constant hypertension without crises.

Pheochromocytoma is most easily diagnosed in the presence of typical crises. A sharp increase in pressure can occur for no apparent reason. Sometimes a crisis is provoked by physical activity, strong emotions, hypothermia, drinking alcohol, or taking certain medications.

This condition is accompanied by:

If the patient is under the supervision of doctors, then during a crisis they take blood for analysis. Signs of the disease: elevated sugar, high levels of leukocytes, lymphocytes, eosinophils.

The crisis ends as quickly as it began. The restoration of normal pressure is accompanied by copious urination. After a crisis, the patient feels tired and overwhelmed. Weakness may persist for several days.

The more often crises occur with pheochromocytoma, the more severe the consequences for the body. Each episode can cause hemorrhage (hemorrhagic stroke, retinal damage, etc.)

Other symptoms of the disease

In addition to arterial hypertension, there are other symptoms of pheochromocytoma. They are more difficult to use to identify a tumor, since they are less specific. If the diagnosis of pheochromocytoma occurs in the later stages, then the patient may already have significant changes in metabolism.

A long-term neoplasm leads to:

weight loss;
blurred vision;
indigestion;
development of secondary diabetes mellitus.

Patients have increased metabolism. They usually have a good appetite, but gradually lose weight. Body weight deficiency can be 10-15%.

If the tumor is malignant, patients are often bothered by abdominal pain of varying intensity.

Diagnosis of pheochromocytoma based on the clinical picture alone is difficult. The disease is confirmed by laboratory tests, pharmacological tests, ultrasound and tomography.

What is pheochromocytoma and how dangerous is it for humans? How to treat this formation? Adrenal pheochromocytoma or chromaffinoma is a hormone-producing tumor that is localized in the adrenal medulla. This formation is formed from chromaffin cells, which produce large amounts of catecholamines - dopamine and. This disease is characterized as a tumor and can be either benign or malignant. Quite often, this formation of the adrenal glands is accompanied by multiple endocrine neoplasia syndrome.

Pheochromocytoma, as confirmed by recent studies, is most often localized in the adrenal medulla (in 90% of all cases). Only in 8% of patients is the tumor located in the area of the aortic lumbar paraganglion. Isolated cases of the development of this formation in the chest, abdominal cavity, pelvis, head, and neck have also been recorded (less than 2%).

This adrenal tumor can occur at any age, but is most often diagnosed in people 25-50 years old. This problem is most common among adult women. In childhood, this tumor is much more often detected in boys. In any case, this pathology is one of those that has a low prevalence among the population. In some cases (about 10%), this tumor becomes familial and is detected in both one of the parents and children. You also need to understand that in 10% of cases this formation becomes malignant. At the same time, metastases from adrenal tumors are very rare. If they develop, then they are detected in the lungs, liver, lymph nodes, muscles, and bone tissue.

When identifying a pheochromocytoma, you can see a formation with a diameter of 1-14 cm, the weight of which is 1-60 g. Adrenal tumors of much larger sizes are also found. Typically this formation has an outer capsule. The adrenal tumor is well supplied with blood. Its hormonal activity depends on its size.

Causes of adrenal tumor formation

The formation of pheochromocytoma, the causes of which are of different nature, occurs in the presence of the following factors:

hereditary factor. The occurrence of an adrenal tumor may be associated with a mutation of certain genes, which provokes negative changes in the adrenal glands;
development of multiple endocrine neoplasia type 2A or 2B. Against the background of this disease, pathological changes occur in the adrenal glands.

Symptoms of an adrenal tumor

In the presence of pheochromocytoma, the symptoms of which manifest themselves quite acutely, many negative processes can be observed in the human body.

This sign is the most characteristic in the presence of an adrenal tumor. There is an increase in both systolic and diastolic values. This symptom can manifest itself in different ways. Some patients experience a persistent increase in blood pressure, but little other signs of the disease appear. Others experience pressure surges of up to 300 mm Hg. Art. (paroxysmal form). Such a hypertensive crisis is accompanied by other symptoms of the disease (pallor of the skin, profuse sweating, intense urination) and disappears quite quickly. Within a few minutes or hours a person may feel well.

The increase in pressure occurs due to the effects of catecholamines produced by the tumor in large quantities. This substance provokes contraction of the smooth muscles that make up the walls of blood vessels. Because of this, the lumen of the arteries and veins is significantly reduced, which leads to increased pressure.

Heart rhythm disturbances due to adrenal tumor

The hormones produced by the tumor act on the adrenergic receptors of the heart. As a result, the frequency and strength of its contractions significantly increases. Subsequently, the opposite effect is observed. When the center of the vagus nerves is excited, the number of heart contractions decreases. This leads to arrhythmia, which is accompanied by the following symptoms:

the patient describes his sensations as a certain feeling of fluttering in the chest and neck;
there is an acceleration of the pulse followed by a slowdown;
there is a feeling that is described as a “failure” in the work of the heart;
pain localized behind the sternum;
muscle weakness;
fast fatiguability;
dyspnea.

Excitation of the nervous system in adrenal tumors

Catecholamines have a stimulating effect on processes occurring in the spinal cord and brain. The transmission of signals from the central nervous system to the organs and tissues of the human body also increases. This is manifested by the following symptoms:

causeless emergence of feelings of anxiety and fear;
chills and trembling throughout the body;
decreased performance, fatigue;
frequent mood swings;
headache (pulsating in nature).

Digestive system dysfunction due to adrenal tumor

Adrenaline, secreted by the hormone producing tumor, stimulates adrenergic receptors located in the intestines. This effect slows down the movement of food through the digestive system and causes the sphincter to contract. If a sick person initially had decreased intestinal tone, then adrenaline, on the contrary, stimulates its peristalsis. This negative impact provokes the following symptoms:

nausea;
abdominal pain accompanied by intestinal cramps;
frequent constipation or, conversely, diarrhea.

Other symptoms of an adrenal tumor

What else is this disease characterized by? If such a tumor is present, the following symptoms are also observed:

activation of the exocrine glands occurs. This phenomenon is accompanied by lacrimation, the release of viscous saliva, and increased sweating;
pallor of the skin is observed, which is explained by vasoconstriction. The skin feels cool to the touch. During a hypertensive crisis, the opposite phenomenon occurs in terms of symptoms - the skin becomes hot and red;
vision deteriorates. Patients often complain of the appearance of dark spots before the eyes;
Increased pressure provokes negative changes in the retina, increasing the risk of hemorrhages. If you undergo an examination by an ophthalmologist, in most cases a change in the fundus of the eye is detected;
development of hyphema. This is a red formation on the white part of the eye;
sudden weight loss of 6-10 kg without changing eating habits. This phenomenon is provoked by the acceleration of metabolism in the body.

Diagnosis of adrenal tumor

If you contact a qualified specialist to determine the condition of the body in the presence of characteristic symptoms, he will be able to make such a diagnosis only after passing a set of tests and undergoing certain procedures. These include:

conducting a conversation with the patient. The attending physician should ask what the person was sick with before and what symptoms bother him. Most patients indicate signs that are characteristic of the presence of such an adrenal tumor;
Every patient with a suspected tumor in the adrenal glands should undergo a diagnostic visual examination, due to which pallor of the skin can be observed. When palpating a formation in the abdominal area, a catecholamine crisis may occur;

stimulation tests. Performed for patients who have persistent high blood pressure. To do this, drugs containing adrenergic blockers (phentolamine, tropafen) are administered intravenously. As a result of this, after 5 minutes the pressure decreases by several units (upper by 40, lower by 25), then we can assume the presence of an adrenal tumor;
general blood analysis. With this disease, its composition changes. In many cases, leukocytes, lymphocytes, and eosinophils increase. This occurs due to contraction of the smooth muscles of the spleen. At the same time, glucose levels also increase;

analysis to determine the concentration of catecholamines in the blood. With pheochromocytoma, diagnosing this negative condition can be difficult, since high levels of hormones (adrenaline, dopamine, norepinephrine, metanephrine and normetanephrine) are determined only within a few hours after a hypertensive crisis. There is no point in doing this analysis later.
examination of the urine of a patient with an adrenal tumor. Laboratory diagnosis occurs on the basis of secretions that are collected within 24 hours or 3 hours after a hypertensive attack. In the presence of the disease, the analysis shows increased levels of catecholamines, protein, glucose in the urine, and the presence of cylinders. The peculiarity of this study is that it should be carried out during an attack. At other times, the test result will be normal;

determination of the level of metanephrines in the blood and urine. During the day after the crisis, the concentration of these substances will be high;
Ultrasound of internal organs. It is necessary for all patients to undergo this study. It allows you to determine where the education is located, what its nature is;
computed tomography of the adrenal glands. It is carried out using a contrast agent, which is administered intravenously, and x-rays. Special equipment takes a series of photographs and then compares them. When using computed tomography, it is very easy to identify all the negative changes in the body, determine the location, size and nature of the tumor;

MRI of organs located in the retroperitoneal space. A modern diagnostic method that does not use x-rays. It allows you to visualize the necessary organs, including the adrenal glands. Using MRI, you can identify tumors of different locations, the size of which is 2 mm;
. It involves intravenous administration of substances that are capable of accumulating adrenal tissue (iodocholesterol, scintadren). After this, the presence of these drugs is recorded using a special scanner. Scintigraphy helps to identify the location of the tumor (not only in the adrenal glands itself, but also in the surrounding tissues), the presence of metastases in the oncological process;
. It is carried out under ultrasound or CT control. Having previously numbed the required area of the body, material is removed from the tumor using a special thin needle. It is examined under a microscope to determine the characteristics of cells and diagnose the presence of malignant processes.

Conservative treatment of adrenal tumors

If pheochromocytoma is present, treatment should be carried out under the supervision of experienced specialists. Drug therapy is aimed exclusively at eliminating the main symptoms of the disease. It is possible to completely eliminate an adrenal tumor only through surgery.

The operation can be performed only after the person’s condition has stabilized. High blood pressure during surgery increases the risk of life-threatening conditions for the patient - uncontrolled hemodynamic syndrome, cardiac arrhythmia, stroke. Therefore, before surgery, it is mandatory to resort to drug treatment, which is aimed at normalizing blood pressure levels.

Depending on the patient’s condition, the use of the following drugs is indicated:

alpha-blockers. Drugs in this group include Tropaphen, Phentolamine. These medications block adrenergic receptors. As a result, they become insensitive to the high level of adrenaline in human blood. When using these drugs, the negative impact of hormones on the human body is reduced, blood pressure is reduced, and heart function is normalized;
beta blockers (Propranolol). This drug reduces the body's sensitivity to adrenaline. When consumed, heart activity is normalized and blood pressure levels are reduced;
catecholamine synthesis inhibitors (Metyrosine). Drugs from this group inhibit the production of adrenaline and norepinephrine in the human body. When taken, the manifestations (by 80%) of all symptoms observed during the disease are reduced;
calcium channel blockers (Nifedipine). Drugs from this group block the entry of calcium into smooth muscle cells in the human body. As a result, vascular spasm is eliminated, which reduces blood pressure and the number of heart contractions.

Surgical treatment of adrenal tumor

After stabilization of the person’s condition, which lasts for at least 5 days, and a complete diagnosis, surgical treatment of the adrenal tumor begins. There are several surgical options, each with its own advantages and disadvantages.

Traditional or open surgery for adrenal tumors

This type of surgery is used by many surgeons to remove adrenal tumors. It involves making a skin incision 20-30 cm long. In this case, the muscles of the anterior abdominal wall, chest and diaphragm are dissected. The process of separating tissue on the way to the adrenal glands can take even the most experienced surgeon from 30 to 40 minutes. After opening access to the adrenal tumor, it is removed, which lasts no longer than 10 minutes. After this, the surgeon performs all actions in the reverse order, suturing all the tissue. This process can take up to half an hour. Such surgical intervention is indicated in cases where it is not possible to accurately determine the location of the adrenal tumor. It has many disadvantages:

The duration of the operation can reach 2.5 hours;
greater tissue trauma, which leads to a long recovery period;
the presence of significant pain after surgery;
The patient is indicated for a long hospital stay in the postoperative period.

Laparoscopic method of surgery to remove an adrenal tumor

How does laparoscopic surgery differ from traditional surgery? During this procedure, a person is placed on a couch on his side, with a cushion placed under his lower back. A small incision is made in the required area, the length of which is about 1 cm. Through it, a special instrument is inserted into the human body - an endoscope with a video camera at the end. Under the control of equipment, the surgeon finds the adrenal gland and removes the tumor, which is usually clearly visible. This operation has many advantages. It is much less traumatic; after it is performed, a small suture remains, and the procedure itself does not last long. Despite these advantages, laparoscopy has many disadvantages:

the procedure is difficult to carry out for patients who have previously undergone surgical interventions on organs located in the abdominal cavity (in the presence of adhesions);
laparoscopy is quite difficult for people with a significant amount of subcutaneous fat;
To perform the operation, carbon dioxide is injected into the abdominal cavity, which creates a working space for the surgeon. As a result, pressure on the diaphragm increases, causing lung volume to decrease. This condition can be dangerous for older people or those with underlying health conditions.

Retroperitoneoscopic surgery to remove an adrenal tumor

It occurs through the lumbar area, where 3 incisions measuring 1-1.5 cm are formed. This operation has many advantages and is considered the best way to remove an adrenal tumor. It is less traumatic, has a small number of complications and minimal recovery time. The patient is discharged 3 days after the operation.

Radical removal of an adrenal tumor has a positive prognosis. The survival rate of patients with benign tumors is 95%, and with malignant tumors – 44%. The relapse rate is 12.5%.

⚕️Melikhova Olga Aleksandrovna - endocrinologist, 2 years of experience.

Deals with issues of prevention, diagnosis and treatment of diseases of the endocrine system: thyroid gland, pancreas, adrenal glands, pituitary gland, gonads, parathyroid glands, thymus gland, etc.

Among all hormone-producing tumors, pheochromocytoma occupies a special place, which not only has a significant effect on many organs and tissues, but also can lead to severe and deadly complications.

The source of the tumor is the so-called chromaffin cells, concentrated mainly in the adrenal medulla. In addition, chromaffin tissue is found along the aorta, in the area of the hilum of the liver and kidneys, in the solar plexus, mediastinum, heart and other parts of the body.

Patients with pheochromocytoma are usually young and middle-aged people, from 30 to 50 years old, more often women, every tenth patient is a child. Up to 90% of cases are neoplasia of the adrenal gland, much less often it grows in the area of the aortic paraganglion, and very rarely in the abdominal cavity, head and neck.

Chromaffin tissue cells produce norepinephrine, adrenaline, dopamine, without which normal functioning of the body, adaptation to stress, maintaining blood pressure, and many metabolic processes are impossible. In a healthy body, they are constantly produced by the adrenal glands, and their concentration increases during stressful situations, injuries and other unfavorable conditions.

The release of adrenaline provokes an increase in blood pressure, frequency and strength of heartbeats, leads to spasm of small blood vessels in the skin and gastrointestinal tract, and an increase in blood glucose levels. Norepinephrine's action is in many ways similar to adrenaline, but it also causes a variety of vegetative symptoms - excitement, fear, tachycardia, sweating, etc. Dopamine is responsible for the psycho-emotional state and vegetative states.

If the effects of these hormones are normally realized under stress and contribute to better adaptation to unfavorable conditions, then in pheochromocytoma their amount is not adequate to the situation. Excess catecholamines provoke hypertension, dystrophic changes in the myocardium and renal epithelium, vasospasm and various autonomic disorders.

Severe symptoms and a high risk of serious complications require early diagnosis and timely treatment, usually surgical, although even after eliminating foci of tumor growth, hypertension and other disorders may not disappear completely.

Causes and essence of pheochromocytoma

The causes of pheochromocytoma are not fully known and remain a mystery in most patients. Approximately 10% of cases are due to chromosomal abnormalities, then they speak of a familial form. The disease is transmitted in a dominant manner, so there may be a significant number of patients among close relatives.

Pheochromocytoma is often combined with other endocrine neoplasms, forming part of the syndrome of multiple endocrine neoplasia. Its growth is possible in neurofibromatosis and medullary thyroid carcinoma. These cases are also familial in nature.

Externally, pheochromocytoma resembles a node surrounded by a capsule, usually measuring within five centimeters, but perhaps more, while the amount of hormones synthesized by tumor cells does not depend on its size. In addition to catecholamines, the tumor can secrete serotonin, calcitonin, and adrenocorticotropic hormone.

Neoplasia that synthesizes predominantly adrenaline is usually dark brown in color, while norepinephrine-secreting tumors are lighter colored (yellow, light brown). The tumor itself has a soft consistency, is rich in blood vessels, and is prone to hemorrhage, which is why it acquires a rather characteristic appearance. Necrosis is possible, after resorption of which cavities remain, and pheochromocytoma has a cystic character.

In the vast majority of cases, the tumor is unilateral; about a tenth are bilateral tumors, and the same amount are extra-adrenal pheochromocytomas. Depending on the characteristics of tumor cells and their behavior, it is customary to distinguish benign and malignant pheochromocytoma, which is also called pheochromoblastoma.

A malignant chromaffin tumor, more often than a benign variety, is localized outside the adrenal glands and synthesizes large amounts of dopamine. It is capable of metastasizing to the lymph nodes, liver, bones and lungs. Metastasis, and not the degree of cell differentiation, is the main prognostic indicator of pheochromoblastoma.

Manifestations of a tumor

Pheochromocytoma is a hormone-producing neoplasia, so its manifestations are associated with the effect of their excess on organs and tissues. Among all such neoplasms, pheochromocytoma has the most serious disorders of blood circulation, heart and kidney function.

Depending on the flow there are several forms of the disease:

Paroxysmal.
Constant.
Mixed.

The paroxysmal form of pheochromocytoma accounts for up to 85% of cases of the disease. It is characterized by hypertensive crises, in which patients complain of severe headache, dizziness, shortness of breath, pain and discomfort in the heart area. Patients look frightened due to a feeling of fear, similar to that that accompanies many heart diseases, trembling, restless, and pale. Sweating appears, the pulse quickens, nausea, dry mouth, convulsions, fever, and the release of large amounts of urine are possible.

In almost all patients, an indispensable manifestation of a crisis is a combination of headache, palpitations and sweating. These symptoms of pheochromocytoma are combined into the so-called Carney triad.

A crisis can be triggered by excessive physical activity, errors in diet, alcohol intake, stress and even urination. You should not try to palpate the tumor yourself, as this can also lead to a sharp rise in pressure.

The crisis can last several minutes, and in severe cases – 2-3 hours or more. Its characteristic feature is its sudden end with the release of a large amount of urine (sometimes up to five liters), profuse sweating, the patient feels tired and very weak.

classic manifestations of pheochromocytoma

The permanent course of pheochromocytoma is characterized by a consistently high level of blood pressure and proceeds similar to primary hypertension. Patients experience weakness, are emotionally unstable, and are prone to metabolic disorders, especially diabetes. At mixed form against the background of chronically high blood pressure, periodic hypertensive crises occur (with excitement, stress and other provoking conditions).

A very dangerous type of tumor progression is considered to be a situation where the pressure is either high or low and changes unpredictably. This phenomenon is called catecholamine shock and is considered a life-threatening condition, which is diagnosed in every tenth patient, somewhat more often in children.

Serious complications of a hypertensive crisis with pheochromocytoma include cerebral hemorrhage, cardiac infarction, pulmonary edema, and acute kidney failure.

It can be difficult to suspect pheochromocytoma due to its atypical course. Thus, a tumor can “simulate” a heart attack, acute pathology in the abdomen, stroke, thyrotoxicosis, kidney disease, and sometimes is completely asymptomatic.

Frequency asymptomatic forms It is difficult to identify tumors, because such patients do not seek help due to the lack of complaints. A case is described where neoplasia reached several kilograms of weight, the patient did not experience any autonomic disorders or hypertension, and the only concern was lower back pain.

Meanwhile, an asymptomatic course is quite dangerous, because the patient can die suddenly from a sudden disruption of hemodynamics or hemorrhage into the tumor. If the tumor is not diagnosed in time, any stress, physical activity, surgery or childbirth can be fatal due to severe hypotension and shock.

Diagnosis of pheochromocytoma

Diagnosis of pheochromocytoma is complex and requires not only a careful assessment of the patient’s complaints and symptoms of the disease, but also a number of laboratory and instrumental tests. A mandatory component is biochemical blood tests to confirm changes in hormonal status.

After examination and conversation with the patient, he is sent for tests, including determination of catecholamines in the blood and urine. If necessary, it is possible to study other hormones (cortisol, parathyroid hormone, adrenocorticotropic hormone). To obtain more reliable results, provocative tests are carried out with the administration of histamine, clonidine, glucagon, etc.

General and biochemical blood tests with a study of sugar levels and calculation of the number of formed elements are required. With a tumor, glucose increases, the numbers of leukocytes and lymphocytes increase, and eosinophils increase.

pheochromocytoma on CT

Among the instrumental methods, ultrasound, computed tomography and magnetic resonance imaging, x-ray examination of the chest organs, and excretory urography are of great diagnostic value. These diagnostic methods make it possible to determine the exact location, size and anatomical features of the tumor formation.

Given the changes in the organ of vision, patients need to consult an ophthalmologist, and the negative impact of the disease on the heart and blood vessels requires regular electrocardiography. If there is doubt about the structure of the tumor, a fine-needle biopsy may be prescribed, but the procedure is used relatively rarely due to the risk of complications.

Video: pheochromocytoma on CT

Treatment

Treatment of pheochromocytoma consists of surgical removal and drug therapy. Since the neoplasm is accompanied by high blood pressure, it is very important to stabilize it at an acceptable level, which will allow the operation to be performed as safely as possible.

Conservative therapy

Drug therapy includes:

Alpha adrenergic blockers (tropafen, phentolamine);
Beta-blockers (propranolol, atenolol);
Calcium antagonists.

The drug phenoxybenzamine, which has adrenergic blocking properties, is quite effective in treating pheochromocytoma. It is prescribed according to a regimen that involves gradually increasing the dosage from 10 mg to 40 mg in two to three doses daily. The dose may be even higher if it ensures stable blood pressure.

During treatment, not only blood pressure is monitored, but also cardiac activity: for two weeks there should be no deterioration in the ST segment and T wave on the ECG, and the number of extrasystoles should not exceed one per 5 minutes of observation.

Catapressan and raunatin can help lower blood pressure during the period between crises in patients with pheochromocytoma, but they do not help against hypertensive crises and only cause a decrease in systolic pressure.

Prazosin and tetrazosin are considered more selective alpha-blockers. Acting specifically on specific receptors, prazosin causes fewer side effects, does not contribute to tachycardia, and a faster action allows you to select an effective dose in a shorter period of time. In addition, when prazosin is prescribed, the likelihood of hypotension in the postoperative period is lower.

For pheochromocytoma with rare hypertensive crises, preference is given to drugs from the group of calcium antagonists - nifedipine, verapamil, diltiazem. These drugs do not contribute to severe hypotension and can even be prescribed for prophylactic purposes if the tumor occurs without hypertension. Beta-blockers are indicated for concomitant heart pathology, severe tachycardia.

It is important to note that the regimen for maintaining optimal blood pressure is selected individually for each patient, taking into account the form of the disease and the characteristics of its course, so there are no identical prescriptions for different patients.

If surgical treatment of adrenal pheochromocytoma is impossible, conservative therapy with drugs that can reduce the amount of circulating catecholamines in the blood is used. The possibilities of such treatment are limited by the high risk of complications and side effects, so it is used quite rarely.

The drug alpha-methyltyrosine can reduce the level of catecholamines by 80%, and its maximum dose should not exceed 4 g. When prescribing this drug, the likelihood of disorders of the digestive tract (diarrhea), as well as neurological and mental adverse reactions, must be taken into account.

Alpha-methylparatyrosine is considered safer, as it sharply inhibits the formation of hormones by the tumor itself and, accordingly, reduces their release into the blood. It can be prescribed in preparation for surgical treatment to reduce the risks of surgery.

In case of malignant transformation of pheochromocytoma or initial growth of pheochromoblastoma, cytostatics are used - vincristine, cyclophosphamide.

Surgery

Surgical treatment consists of removing the adrenal gland with the tumor. Its difficulties are due to the need for extremely careful manipulations in the surgical field, since even touching the tumor can provoke a sharp release of hormones. It is important to provide free access to the affected tissues and the ability to view internal organs to exclude extra-adrenal foci of tumor growth. To remove pheochromocytoma, transperitoneal, extraperitoneal, through the chest cavity or a combined approach is used.

With multiple pheochromocytoma, the situation is even more complicated. If other tumor foci are found, they are removed as much as possible. If a one-step operation is not possible, treatment is carried out in several stages. An extreme case is considered to be the abandonment of ectopic tumor tissue, for which there must be good reasons - too high a risk for the patient or lack of technical ability to remove the tumor.

The operation itself consists of laparotomy access to the tumor (laparoscopy is not used due to possible complications) and removal of it along with the adrenal gland (total adrenalectomy). In case of bilateral damage, the surgeon excises both adrenal glands, and if other growth foci are detected, he removes them as well. The operation is carried out under careful monitoring of blood pressure and other hemodynamic parameters.

adrenalectomy - surgical removal of the adrenal glands

If the patient is a pregnant woman, then pheochromocytoma “will not allow” to carry and give birth to a child without a huge risk to the health, first of all, of the expectant mother. In such cases, doctors have no other choice but to terminate the pregnancy (abortion or long-term caesarean section) and then remove the tumor.

Video: example of laparoscopic surgery to remove pheochromocytoma

After surgical removal of pheochromocytoma, some complications are possible. If the pressure does not drop, but continues to remain high, then there is a possibility of a violation of the operating technique when the renal artery was accidentally ligated. Another reason may be the persistence of areas of pheochromocytoma, especially with multicentric growth, as well as the presence of primary hypertension as a concomitant disease.

Severe hypotension is another possible complication of treatment, the causes of which may be internal bleeding, insufficient control of circulating blood volume, or the continued effect of antihypertensive drugs prescribed in the preparatory period or during the operation itself.

For benign pheochromocytoma, surgical treatment leads to an almost complete cure for most patients. There is no need to talk about complete healing, since the tumor significantly changes the condition of other organs. The probability of recurrence does not exceed 12%, and in the case of unilateral lesions the risk of involvement of the second adrenal gland is very small. A poorer prognosis for patients with multiple pheochromocytoma, especially if the surgeon was unable to excise all foci of the tumor.

In the postoperative period, some tumor symptoms may persist. This is due to the long-term effect of catecholamines on the heart and kidneys, which have already undergone secondary changes. In this regard, persistent hypertension and/or tachycardia are considered as consequences of the presence of a tumor, and they may appear some time after the operation.

To summarize, I would like to note that surgery is not only the most radical method of treatment, but also the only way to save the patient’s life, so in no case should it be abandoned. Even if it is not possible to completely remove the tumor tissue, and hypertension still develops in the postoperative period, these consequences are incomparably safer than the presence of pheochromocytoma in the adrenal glands.

The author selectively answers adequate questions from readers within his competence and only within the OnkoLib.ru resource. Face-to-face consultations and assistance in organizing treatment are not provided at this time.

The adrenal glands are endocrine organs that take part in normalizing metabolism and adapting the body to stressful situations. Pheochromocytoma of the adrenal gland interferes with the performance of its direct functions. Whatever the nature of the tumor, under its influence the production of hormones is disrupted, which negatively affects the functioning of the entire body. How to recognize the development of the disease and how to treat it?

Causes

Pheochromocytoma is a neoplasm that arises in the adrenal medulla. During its development, it releases adrenaline and norepinephrine into the blood. There are not many reasons for the development of this disease:

A tendency to the disease, transmitted by heredity in 10% of cases, if one of the person’s relatives had this disease, it can also occur in him.
Hereditary syndromes caused by atypical proliferation of endocrine gland cells, including Sipple syndrome and Godlin syndrome. Affects the adrenal glands. It is also transmitted by heredity.

But most often, the development of pheochromocytoma does not depend in any way on diseases in the family, and it occurs spontaneously. Risk factors that may provoke this cancer are: ionizing radiation, constant stress, mutagenic chemical environmental factors. Such a neoplasm is a fairly rare occurrence, but can appear in both adults and children (10% of cases). It most often occurs in people between the ages of 20 and 50 and is more likely to occur in women. Among children, it is found more often in boys.

Types of neoplasm

Pheochromocytoma detected in a patient is always a reason for surgery to remove the adrenal gland, including due to the risk of malignancy.

Depending on where it is located, there are the following types of pheochromocytomas:

arising on the adrenal gland;
developing outside the adrenal gland.

The tumor may have one focus of development or several. Also, its development is possible both on one of the adrenal glands and on two at once (10−15% of cases). The nature of pheochromocytoma can be either benign or malignant. The neoplasm degenerates into cancer with a 10% chance. If this happens, the course of the disease is aggravated, pheochromocytoma gives metastases, since pathological cells are spread through the blood and lymph throughout the body. Metastases are found in lymph nodes, liver parenchyma, skeletal bones, brain and lungs.

Tumor structure

Pheochromocytoma has good blood circulation and grows in diameter to 10-12 centimeters. The tumor will weigh no more than 60-70 grams. A benign neoplasm is contained in a dense capsule. Metastases and pheochromocytoma of a malignant nature do not have a clearly defined membrane. The structure of pheochromocytomas contains chromaffin cells with varying degrees of disruption of cellular processes.

Symptoms of the disease

Symptoms characteristic of the development of the disease include:

increased blood pressure in the vessels;
increased heart rate;
active sweating;
severe pain in the head;
attacks of involuntary muscle contractions;
paleness of the skin;
shortness of breath.

Blood pressure with a tumor can increase to 200 or more mmHg.

There are also more general symptoms of pheochromocytoma, which can also be observed not only when a neoplasm occurs: restless nervous state, pain in the stomach, problems with the excretory system, weight loss. With pheochromocytoma, they appear briefly and soon disappear; a person can experience this condition a couple of times a day. Such symptoms occur due to stress on the body - physical or emotional.

Diagnostic measures

Taking tests and carrying out diagnostic procedures for suspected pheochromocytoma is done several times. The patient is examined in a calm state and after a recent attack of exacerbation of symptoms. After a spontaneous exacerbation, adrenaline, norepinephrine, dopamine, as well as their breakdown products, enter the blood. They are also found in urine. The presence of a pathological change is determined by the excess amount of these substances in the samples.

Laboratory research

Tests are carried out within several hours after the attack.

A course of research aimed at studying the condition of the patient’s body includes:

Study of blood composition: a general analysis that will show an increased amount of all blood components, determination of the amount of catecholamines in the blood structure.
Urine tests: per day and every 3 hours.

Instrumental diagnostic methods

An instrumental examination involves studying the condition of the human adrenal glands and includes:

Ultrasound diagnostics (ultrasound) - reveals the location of adrenal pheochromocytoma and its size.
Computed tomography (CT) - determines the nature of the development of the tumor, and is necessarily performed before surgery.
Magnetic resonance imaging (MRI) - shows the adrenal glands in combination with the abdominal space around, clarifies the size of the pheochromocytoma and the nature of its development, if CT is not indicative.
Centigraphy - detects neoplasms both in the adrenal glands and in neighboring organs and tissues.
Fine needle aspiration biopsy - performed if CT and MRI did not reveal anything and has the same purpose.

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

Pheochromocytomas most 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 excess amounts of peptides and biogenic amines (dopamine, adrenaline and norepinephrine), resulting in catecholamine crises.

Malignant tumor types (pheochromoblastomas) account for less than 10% of the total number of diagnosed pheochromocytomas. It is very common for such tumors to be located outside the adrenal glands. Secondary foci (metastases) form in regional lymph nodes, liver, distant organs (lungs) and tissues (muscle and bone).

Etiology

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

Every tenth patient has a genetic predisposition. During the history taking, it turns out that these tumors were previously diagnosed in the parents. Genetics believe that the 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 originate not only from the adrenal medulla, but also from the aortic lumbar paraganglion (in such cases they are called paragangliomas). Neoplasms of this type are also detected in the pelvic, thoracic and abdominal cavities. In rare cases, chromaffinoma is found in the head and neck area. Cases of tumor localization in the pericardium and cardiac muscle have been described.

Active substances that can be synthesized by chromaffin:

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 tumor (they are variable and can reach 5 cm). The average weight of pheochromocytoma is 70 g. These encapsulated tumors are characterized by rich vascularity.

Symptoms of adrenal pheochromocytoma

Symptoms are determined by an excess of a particular substance. Hypersecretion of catecholamines by pheochromocytoma leads to the development. Tumors are detected in approximately every hundredth patient with constantly elevated 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 crisis with pheochromocytoma:

up to 200 mm. rt. Art. and more;
unmotivated feeling of anxiety and fear;
intense;
pale skin;
(excessive sweating);
cardialgia();
cardiopalmus;

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

The duration of the paroxysm ranges from several minutes to an hour or more. Their frequency varies from single attacks over 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 experiences heavy sweating and increased urine output (up to 5 liters) with low specific gravity. He complains of general weakness and a feeling of “brokenness” throughout his body.

Factors that can provoke a crisis:

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

The most severe outcome of an attack is catecholamine shock. It is characterized by uncontrolled hemodynamics - episodes and hypertension change randomly and are not amenable to drug correction. In severe hypertensive crisis caused by chromaffinoma, complications such as dissection and the development of functional are not excluded. Retinal hemorrhages are often observed. Paroxysms pose a great danger to women during pregnancy.

With a stable course, the patient has consistently high blood 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 cephalgia and increased physical and mental fatigue.

Metabolic disorders in particular include increased blood glucose levels (hyperglycemia), which often causes the development of diabetes.

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

Diagnosis of pheochromocytoma

During a general examination, patients are diagnosed with 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 stands up, the pressure drops sharply).

Important:palpation examination (palpation) of the 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 level of chromogranin-A (universal transport protein), adrenocorticotropic hormone, calcitonin and trace elements - calcium and phosphorus - is also determined in the serum.

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

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

A significant proportion of subjects are found to have hypertension-related vascular damage to the retina (retinopathy). All patients with suspected pheochromocytoma should undergo additional examination by an ophthalmologist.

During the differential diagnosis of 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, ultrasound scanning and tomographic examination (and) of the adrenal glands are considered the most informative. They allow you to clarify the size and location of the tumor. Additionally, they resort to selective arteriography of the adrenal glands, as well as the chest organs (to confirm or exclude the intrathoracic localization of chromaffinoma).

Pathologies for which differential diagnosis is carried out:

paroxysmal;
some types.

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

It is not always possible to reliably establish the malignancy of a tumor at the preoperative stage. We can 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 pheochromocytomas are detected, drug therapy is carried out, the purpose of which is to reduce the severity of clinical symptoms and relieve paroxysmal attacks. Conservative measures involve prescribing drugs from the group α-blockers(Phentolamine, Phenoxybenzamine, Tropaphen, 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 reducing catecholamine levels is A-methyltyrosine, but its regular use can provoke mental disorders and digestive disorders.

Then surgical intervention is performed - total adrenalectomy. During the operation, the affected adrenal gland is removed along with the tumor. Since there is a high probability of pheochromocytoma being located outside the adrenal gland and the presence of multiple neoplasms, preference is given to the “classical” laparotomy approach, but a less traumatic one is also possible.

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

When diagnosing pheochromocytoma 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 levels normalize, and other clinical signs regress. If hypertension persists, there is reason to assume the presence of ectopic tumor tissue, incomplete removal or accidental damage to the renal artery.

A unified standard for surgical interventions for multiple tumors has not been developed. Sometimes it is considered appropriate to perform 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 approximately 12% of cases. All patients who have undergone surgery are strongly recommended to be examined annually by an endocrinologist.

Plisov Vladimir, medical observer