Pheochromocytoma is a hormonally active tumor composed of adrenal medulla or extraadrenal chromaffin tissue. Most often, the tumor is localized in one of the adrenal glands, is benign.

The reasons

The causes of the disease are unknown, but there is an opinion about the genetic nature of pheochromocytoma.

The disease occurs at any age, but more often at 25-50 years. This tumor often develops in middle-aged women. In childhood, pheochromocytoma is more common in boys. In general, this tumor is not common enough.

About 10% of cases of the disease occur in the familial form, when the disease occurs in the presence of the same disease in at least one of the parents, regardless of gender.

A hereditary disease is also rare, in which the development of tumors in different endocrine glands is observed. In this case, pheochromocytoma can be combined with thyroid cancer, adenoma within the thyroid glands or tumors of the mucous membranes, intestines (adenoma is a benign tumor of the gland tissue).

Malignant pheochromocytoma is in 10% of cases, but metastases are not common.


Manifestations of the disease are due to excessive secretion of catecholamines. Adrenal pheochromocytoma secretes epinephrine and norepinephrine, while extra-adrenal tumors secrete only noadrenaline, which has a much lesser effect on metabolism. This means that the tumor, which is located in the adrenal glands, is traditionally manifested by a large number of symptoms.

The most constant symptom of pheochromocytoma is arterial hypertension (increased blood pressure), which occurs most often with periodic hypertensive crises. During the crisis, there is a sharp increase in blood pressure (BP), and in the inter-crisis period, blood pressure returns to normal. A somewhat less common form is characterized by a constant increase in blood pressure, against which crises develop. In addition, pheochromocytoma can proceed without crises with stably elevated blood pressure.

Hypertensive crises are often accompanied by neuropsychiatric, metabolic disorders, gastrointestinal symptoms and blood disorders (paroxysmal form of the disease). During an attack, a feeling of fear, anxiety, trembling, chills, pallor of the skin, headache, pain behind the sternum, pain in the heart area, palpitations, heart rhythm disturbance (which the patient evaluates as a feeling of a sudden “failure”), nausea, vomiting, increased body temperature, sweating, dry mouth. It is not necessary that all of these symptoms appear, they can vary. There are changes in the blood test: an increase in the content of leukocytes, lymphocytes, eosinophils, an increase in the content of glucose (sugar).

The crisis ends as quickly as it begins. Blood pressure returns to its original values, the pallor of the skin is replaced by redness, sometimes there is profuse sweating. Up to 5 liters of light urine is excreted. After an attack, general weakness and weakness persist for a long time.

In severe cases, the crisis can be complicated by retinal hemorrhage, cerebrovascular accident (stroke), and pulmonary edema.

Attacks appear, as a rule, suddenly and can be provoked by hypothermia, physical or emotional stress, sudden movement, alcohol or certain drugs. The frequency of attacks is different: from 10-15 crises every day to one for several months. The duration of the attack is also not the same – from several minutes to several hours.

With a stable form of the disease, constantly high blood pressure is noted, there may be violations of the kidneys, changes in the fundus (which the oculist can tell about). High excitability, mood variability, fatigue, headache are observed.

Symptoms such as palpitations, sweating, diarrhea (diarrhea), weight loss that occur with pheochromocytoma are a manifestation of increased metabolism due to the production of adrenaline by the tumor and are not associated with impaired thyroid function (which has similar manifestations). In addition, a violation of carbohydrate metabolism often develops, which manifests itself in an increase in blood glucose levels.

With a malignant tumor – pheochromoblastoma – significant weight loss, abdominal pain are not uncommon. Development of a diabetes mellitus is possible.


Laboratory data: in the blood during an attack, leukocytosis, transient lymphocytosis and eosinophilia are noted. There may be erythrocytosis and a high erythrocyte sedimentation rate, hyperglycemia, an increase in the concentration of NEFA, an increase in the fibrinolytic activity of the blood and its coagulability. In a three-hour portion of urine collected after a crisis, the content of catecholamines is 2-3 times or more higher than their daily excretion.

In the urine – transient proteinuria, not often glucosuria, cylindruria.

For the diagnosis of pheochromocytoma, the content of catecholamines in the blood is determined. In sick patients, the daily excretion of catecholamines increases sharply, and when the tumor is localized in the adrenal gland, it is mainly due to adrenaline (more than 50 μg), while in extra-adrenal localization it is due to norepinephrine (more than 150 μg).

Urinary excretion of the metabolite of catecholamines, vanillylmandelic acid, is also determined, which increases 2-10 times (normally not) – up to 10 mg per day.

In all cases of arterial hypertension, especially in young people, in the absence of convincing evidence of primary kidney damage, functional tests are performed using α-blockers (regitin, tropafen), which are indicated in the presence of persistent hypertension.

Test with regitin. With the introduction of / in 5 mg after 5 minutes, the systolic pressure drops by 35 mm Hg. Art., and diastolic – 25 mm Hg. Art. With intramuscular injection of 10 mg after 20 minutes, blood pressure decreases.

Histamine test. With intravenous administration of 5 centi-mg, blood pressure rises after 2-3 minutes, especially if the crisis is stopped by the subsequent administration of α-blockers (initial pressure is not more than 150/100 mm Hg, an increase is more than 50/40 mm Hg. Art.). The test with histamine is used in the interictal period, it is advisable to combine it with the determination of catecholamines in the urine.

It is also carried out:

    • Ultrasound of the abdominal organs;
    • aortography;
    • scintigraphy;
    • computed tomography (most effective).


Recovery can occur only after surgical removal of the tumor. Mortality during surgery – 1.3%. If surgical intervention is improbable, regitin is injected intramuscularly at a dose of 10 mg 4 times a day.

In the last time, a product from the α-methyl-L-tyrosine group (Demsec, Metyrosine) appeared, which is used 1-2 tablets every day.

In case of a pheochromocytoma crisis, α-blockers are injected intravenously (2–4 mg of phentolamine hydrochloride or 1–2 ml of a 2% solution of tropafen in an isotonic sodium chloride solution. The introduction of products is repeated if necessary, and then injected intramuscularly switching to oral administration (20-50 mg of phentolamine).In severe tachycardia (more than 120 beats per minute), not often with rhythm disturbance, beta-blockers are prescribed.If a state of uncontrolled hemodynamics develops, emergency surgery is indicated – removal of the tumor.

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