August
12
19:53
Endocrinology

Hormonally active adrenal tumors: aldosteronoma, pheochromocytoma

hormonally active adrenal tumors are a group of tumors that cause increased secretion of glandular cells of a particular hormone.Since each hormone has a number of inherent in it only effects the tumor that causes overproduction of the hormone, appears complex of symptoms that indicate improvement of a specific hormone (or several) in the body.This article will talk about the diagnosis and treatment of two kinds of hormonally active adrenal tumors aldosteromas and pheochromocytoma.

Content

  • 1 aldosteroma or Conn's syndrome
    • 1.1 Clinical manifestations aldosteroma
    • 1.2 Diagnostics aldosteroma
    • 1.3 Treatment aldosteroma
  • 2 Pheochromocytoma
    • 2.1 causes and mechanisms of disease
    • 2.2 Pathology pheochromocytoma
    • 2.3 Clinical manifestations of pheochromocytoma
    • 2.4 diagnosis of pheochromocytoma
    • 2.5 treatment of pheochromocytoma
    • 2.6 forecast

There are 5 main types of hormonally active adrenal tumors:

The fact that the first three are the type of tumor, we talked

in the first part of this article.Here we will focus on hormone-producing tumors of the adrenal glands as aldosteronoma and pheochromocytoma.

Hormonally active adrenal tumors: aldosteronoma, pheochromocytoma

disease caused by an increase in blood levels of the hormone aldosterone, in fact, several.Depending on the causes of its increase, hyperaldosteronism is divided into primary (independent of the renin-angiotensin system) and secondary (dependent on it).

Normally production of aldosterone is regulated by a system called the renin-angiotensin-aldosterone cascade.Under the influence of certain factors triggered a series of biochemical processes that stimulate the production of the kidneys and other organs of special substances - renin, which contributes to the formation of angiotensin, from which later formed aldosterone.

Hormonally active adrenal tumors: aldosteronoma, pheochromocytoma In primary hyperaldosteronism increase the level of aldosterone is independent of the processes of formation of renin and angiotensin - is observed at local disease of the adrenal glands, the tumor malignancy.

Secondary hyperaldosteronism occurs with serious heart disease, liver and kidneys, as well as a result of taking diuretic drugs.

Almost 2/3 of primary aldosteronism occurs because of the small size (no more than 4 cm) unilateral adenoma of the adrenal cortex.The malignant form of primary hyperaldosteronism diagnosed in only 2% of cases and is a carcinoma.The remaining 30% of cases of primary hyperaldosteronism associated with an increase in the number of glandular cells - hyperplasia - the adrenal cortex.The causes of this condition is unknown, however hyperaldosteronism in this case is called idiopathic.

Hormonally active adrenal tumors: aldosteronoma, pheochromocytoma Elevated blood levels of aldosterone causes a delay in the body of sodium and increases the excretion of potassium in the urine.Excess sodium entails a delay in the body fluid, which leads to increased blood volume, inhibits synthesis of renin and angiotensin, increases the susceptibility of the vessel walls to adverse factors causing increased blood pressure.Potassium deficiency adversely affects the functioning of many organs and systems, in particular, it causes disruption of the structure and function of the kidney tubules, muscle, nervous system (both central and peripheral), amplifies neuromuscular excitability reduces glucose tolerance.

Suffer aldosteroma usually those aged 30-50 years and women - 3 times more often than men.

Patients may complain of:

Patients with symptoms typical of aldosteronism, shows a number of diagnostic procedures that allow accurate diagnosis and differential diagnosis of primary and secondary hyperaldosteronism.

First of all, patients complain of persistent high blood pressure and other symptoms of the above, prescribed general and biochemical blood tests, urinalysis and ECG.

In general, the analysis is determined by elevated erythrocyte sedimentation rate, may experience symptoms of anemia (decrease in hemoglobin and red blood cells) and increasing the level of white blood cells, particularly neutrophils.

In the biochemical analysis of blood parameters define the content of potassium and sodium.Sharply reduced level of the first, second, on the contrary, increased.

The urinalysis showed a decrease of its density, as well as an excess of potassium.

cardiogram reflect measurements specific to reduce the content of potassium in the blood, namely:

Hormonally active adrenal tumors: aldosteronoma, pheochromocytoma

When detected in the blood of hypokalemia and hypernatremia, in the case of an adequate supply of these minerals in the diet, determine blood levels of hormones - aldosterone and renin.It found that the content of aldosterone increased dramatically - in 3-4 times higher than normal values.The level of renin dependent on what kind of aldosteronism occurs in this case - primary or secondary.In primary aldosteronism renin concentration in blood plasma is lowered, and in the case of secondary where increased aldosterone levels associated with any serious background pathology, renin activity is increased in parallel with an increase in aldosterone levels.

Excretion (selection) aldosterone excretion will also be enhanced.

for the differential diagnosis of primary and secondary hyperaldosteronism can be carried out and functional tests - for example, with spironolactone or furosemide.

In order to confirm the diagnosis methods used topical diagnosis - computer and magnetic resonance imaging.Apparatus CT reveals adrenal tumors larger than 10 mm, and even MRI detects a tumor of less than 10 mm in diameter.

ultrasound adrenal little information, because it can detect a large tumor - the size of more than 2-3 cm.

patients suffering from idiopathic form of primary aldosteronism resulting from diffuse bilateral adrenal hyperplasia, is a conservative therapy with the drug spironolactone in combination with medicinalDecreasing blood pressure.

movie about aldosteroma:

Pheochromocytoma - a cancer of the adrenal medulla, namely the so-called chromaffin tissue, producing substances catecholamines - adrenaline, noradrenaline, dopamine.The second name of this formation - hromaffinnoma.

Hormonally active adrenal tumors: aldosteronoma, pheochromocytoma

important to know that in the process of malignant transformation chromaffin tissue in addition to catecholamines can be produced in large quantities, and other substances, such as serotonin or adrenocorticotropic hormone: it contributes to the clinical picture of the disease much variety and difficult to diagnose.

Unfortunately, about 60% of pheochromocytomas are diagnosed posthumously.In 9 of 10 patients the tumor site characteristic - the adrenal glands (usually one, sometimes on both sides), and 10% of patients the tumor is localized in certain areas of the body.The most common places extraadrenal chromaffin tissue localization are:

Usually pheochromocytoma - a benign tumor, but there is a malignant form of it, called feohromoblastomy.

Unfortunately, we can not say reliably why there pheochromocytoma.Perhaps there is a genetic predisposition to this disease, since it is often a family history of the disease it.

mechanism of hromaffinnomy associated with increased production of tumor cells catecholamines - epinephrine, norepinephrine and dopamine.At the time of crisis the level of these substances in the blood increases significantly - often higher than normal concentration tenfold.

main effects of catecholamines is high blood pressure and an intensification of metabolic processes.Under the influence of these hormones there is a sudden sharp spasm of blood vessels and increases heart rate.These changes lead to a sharp increase to high numbers in both systolic and diastolic blood pressure.Spazmiruyutsya not only large vessels, but also vessels of the skin and mucous membranes, abdominal muscles.The lumen of the bronchi and intestine expands, the process of splitting of glycogen to glucose in the liver - is accelerated.Changes in the myocardium characterized by the development of catecholamine myocardiodystrophy and arrhythmias.Accelerate metabolism, heat production, glycogen and fat breakdown, weaken the effects of insulin.

benign pheochromocytoma usually has unilateral location and formation is not more than 5 cm in diameter and 100 g in weight.It grows slowly, invasive growth is not typical.

feohromoblastomy often reach enormous sizes - up to 30 cm in diameter and weighing 2 kg or more.It is soldered to surrounding tissues, coated capsule having an uneven thickness.It is characterized by rapid growth, grows into the surrounding organs and tissues through the blood and lymph spreads.Location of the tumor is usually bilateral.Can both be present not one, but multiple tumors.

There is a malignant form of conditional feohromoblastomy.This tumor is in its micro and macro characteristics occupies an intermediate position between benign and malignant forms.This option is most common pheochromocytoma.

pheochromocytoma may be accompanied by hyperplasia of the adrenal medulla.

classical clinical picture is peroksizmalnaya pheochromocytoma (attacks) hypertension in conjunction with metabolic and autonomic symptoms such as:

Depending on the characteristics of the disease distinguish 4 forms of pheochromocytoma.

attack is usually preceded by "aura" as paresthesia, muscle tremor, abdominal pain shooting character.After that appear and grow rapidly headache, compressive nature in the heart, heartbeat.Also, there may be sick "flicker" and double vision, pain in the lower back and abdomen.The patient is excited, experiencing agonizing fear of death, screaming.The skin of his pale, covered with cold sweat.All these changes are taking place against a background of high blood pressure to the level of 280-360 / 180 mm Hg.Art.Pulse aritmichen.The attack marked the end of the redness of the skin, profuse sweating, salivation and lacrimation, nausea, vomiting, copious, expressed general weakness.With progression of the classical form of the disease in the cardiovascular system and the kidneys appear secondary changes, entailing a steady rise in blood pressure.

If pheochromocytoma is available for pregnant women, it can simulate the late preeclampsia - eclampsia up to, or combined with it.In the absence of timely diagnosis in 50% of cases, and the mother and the fetus die.Diagnosed as pheochromocytoma during pregnancy is a direct indication for its interruption or premature delivery.

Patients childhood hromaffinnoma proceeds without typical for her paroxysms, and is characterized by persistent high blood pressure.Also, children disturbed vision: when viewed from an ophthalmologist retinal hemorrhage determined.

clinical picture described above, allows the physician to suspect the disease.To confirm the diagnosis carried out biochemical analysis of blood, determining the level of adrenaline, noradrenaline and dopamine.When pheochromocytoma content of these substances in the blood can increase even ten times.

on higher levels of catecholamines in the urine also increased their content, and their metabolic products - vanillylmandelic acid (WFC).The condition is to collect urine or within the first three hours after a crisis, or for the day.

to put an accurate diagnosis, the above studies should be repeated 3-4 times.

can be carried out and functional tests with histamine, glucagon (these substances trigger the release of adrenaline by the adrenal glands) or tropafenom, regitinom, clonidine (block the vasoconstrictor effects of catecholamines).

In addition, if at the time of the attack to take a blood test, it found an increase in white blood cell count (leukocytosis) and glucose.

the purpose of topical diagnosis, as in other tumors of the adrenal glands, using a computer or magnetic resonance imaging.With the localization of adrenal pheochromocytoma is conducted scintigraphy (scanning) with a preliminary introduction into the body substances accumulating in chromaffin tissue, or angiography.US in this case, little information.

Hormonally active adrenal tumors: aldosteronoma, pheochromocytoma main method of treatment is surgery hromaffinnomy in the amount of unilateral adrenalectomy (removal of the adrenal gland from injury), together with the new formation.The criterion for the complete removal of the tumor is the absolute regression of symptoms and normalization of the level of catecholamines in the blood.

When adrenal Stroke patient must enter adrenolytics - phentolamine.It is administered in a specific dosage of 5-10 minutes to complete relief of crisis.Upon reaching the target the same dose administered 2-4 times per one hour for 24 hours and then transferred to the patient immediately prior to oral surgery.If the intake adrenalitika saved palpitations or arrhythmia, the treatment was added β-blocker.

As mentioned earlier in this article, 60% of patients die without knowing their diagnosis.The causes of death are, as a rule, severe vascular complications, strokes and heart attacks on a background of uncontrolled hypertension.

With timely surgical treatment of five-year survival is celebrated in more than 95% of cases.Sometimes - every tenth patient - unfortunately, the case of relapse.