Aldosteroma – gormonalno the active tumor of a glomerular epithelium of bark of adrenal glands leading to development of a clinical syndrome of Conn - primary aldosteronizm. Distinguish from symptoms of an aldosteroma cardiovascular (arterial hypertension, headaches, a kardialgiya, sight violation), neuromuscular (a myopathy, a mialgiya, paresteziya, spasms), kidney (a polyuria, a polidipsiya, an izostenuriya). The diagnosis of an aldosteroma is established on a characteristic clinical picture of a disease, results of laboratory analyses and tool researches: Ultrasonography, stsintigrafiya, KT (MPT), angiography and selective venografiya of adrenal glands. At an aldosteroma radical removal of a tumor with the affected adrenal gland is shown (adrenalektomiya).
The Simptomokompleks caused by the raised production of mineralokortikoidny hormone of an aldosteron was for the first time described by D. Conn, and received the name of "primary aldosteronizm" or Conn's syndrome. In 70-85% of cases adenomas of adrenal glands, in other cases – a giperplaziya of bark of adrenal glands, tumors of a thyroid gland or ovaries having hormonal activity are the reason of primary giperaldosteronizm.
Under aldosteromy in endocrinology understand aldosteronsekretiruyushchy adenoma of adrenal glands which development is followed by signs of primary aldosteronizm. Aldosteroma in most cases have good-quality character, less than in 5% of cases – malignant. Aldosteroma, as a rule, comes to light aged from 30 up to 50 years, and at women by 3 times more often than at men. Cases of development of an aldosteroma at children's age are described.
Reasons and pathogenesis of an aldosteroma
The causes of an aldosteroma, as well as many other tumoral formations of bark of adrenal glands, are authentically unknown. Presumably a part in its development is played by heredity.
Aldosteroma is shown autonomous superfluous (increased by 40-100 times) by secretion of mineralokortikoidny hormone – the aldosteron regulating water and electrolytic exchange in an organism. High level of an aldosteron leads to strengthening of a reabsorption of ions of sodium in kidney tubules and the raised ekskretion of ions of potassium, magnesium and hydrogen with urine that promotes a delay of liquid, a gipervolemiya, a gipokaliyemiya and a metabolic alkaloz, pathological changes in various bodies and systems. Feature of primary aldosteronizm at an aldosteroma is low activity of a renin in blood plasma.
The good-quality aldosteroma represents small (no more than 1-3 cm) the tumor of an adrenal gland of yellowy-brown color surrounded with the thin soyedinitelnotkanny capsule. The good-quality aldosteroma can be combined with an atrophy or a giperplaziya of the zones of bark of adrenal glands surrounding it. Primary malignant aldosteroma developing from own elements of bark of adrenal glands is characterized by rapid growth, the big size and weight; sometimes at the small amount of education there can already be metastasis signs. Aldosteroma more often happen single (to 70-90% of cases), in 6% of cases – multiple to bilateral localization. Morphologically aldosteroma have a non-uniform structure: can consist of the cages similar to cages of a puchkovy or mesh zone.
Clinical manifestations of an aldosteroma are caused by the violations connected with primary aldosteronizm and presented by three main syndromes - cardiovascular, neuromuscular and kidney.
The cardiovascular syndrome at an aldosteroma is caused, mainly, by a delay of sodium and water, gipervolemiy, development of hypostasis of an internal cover of a vascular wall (intims) and narrowing of a gleam of vessels, increase in peripheral resistance, increase in reactivity of vessels on action of pressor factors, in particular, an aldosteron. The Yoklinichesky picture of an aldosteroma is characterized by the constant moderate or expressed arterial hypertension, a headache, development of changes of an eye bottom (a hypertensive angiopatiya, angiosklerozy, a retinopathy and a neuroretinopathy), a kardialgiya, a hypertrophy, and further - dystrophy of a myocardium of the left ventricle.
The neuromuscular syndrome is connected with deficiency of potassium and magnesium, giperkhloremichesky acidosis, dystrophic changes of muscular and nervous tissue. At an aldosteroma it is shown by fatigue, muscular weakness of various degree of expressiveness, locks, pains in fingers of hands and feet, gastrocnemius muscles, is frequent - paresteziya and spasms. At an aldosteroma the gipokaliyemichesky crises which are followed by a sharp headache, vomiting, short wind, decrease (loss) in sight, a mioplegiya, sometimes approach of sluggish paralysis or convulsive attacks, complicated by development of sharp coronary insufficiency, sharp violation of brain blood circulation (stroke) can be observed.
At an aldosteroma the kaliyepenichesky nephropathy which is shown violation by concentration ability of kidneys, thirst, a plentiful and frequent urination (a daily diuresis to 10 liters), a nikturiya, izostenuriy develops. Peripheral hypostases are not characteristic of an aldosteroma. At the expressed chronic gipokaliyemiya excitability of a myocardium, insulin secretion by b-cells of a pancreas and tolerance to glucose are broken.
At malignant aldosteroma along with the main symptoms there can be belly-aches, temperature increase of a body and other symptoms of intoxication. About 10% an aldoster proceed asymptomatically.
Diagnostics of an aldosteroma
Diagnostics of an aldosteroma is based on characteristic clinical manifestations of a syndrome, results of laboratory analyses, functional tests, tool researches. In 2 weeks prior to inspection it is desirable for patient to stop reception of hypotensive medicines. Ultrasonography of adrenal glands and radio isotope scanning (stsintigrafiya) of adrenal glands are used for identification of the available pathological changes and specification of their character (a giperplaziya, a tumor), by KT of adrenal glands and MRT of adrenal glands - for definition of localization and size of an aldosteroma.
In the general analysis of urine at an aldosteroma the low relative density and alkaline reaction, a proteinuria, increase in a daily ekskretion of potassium and an aldosteron comes to light. Biochemical blood test finds a gipernatriyemiya, a gipokaliyemiya, high basal level of an aldosteron in serum, decrease of the activity of a renin of plasma, gipokhloremicheskiya . For the purpose of diagnostics of primary aldosteronizm at an aldosteroma carry out test with spironolaktony, test with loading a hydrochlorothiazide, "mid-flight" test.
Radiological methods of diagnostics of an aldosteroma - the pnevmosuprarenografiya and an angiography of adrenal glands can yield inexact results because of the small sizes of a tumor and its bad vaskulyarization. The selective venografiya of adrenal glands with simultaneous determination of levels of an aldosteron and cortisol in blood of nadpochechnikovy veins is the most informative though its carrying out is technically difficult and fraught with complications. Repeated increase in a ratio / cortisol is characteristic of an aldosteroma.
Differential diagnostics of an aldosteroma is carried out from diffusion melkouzelkovy giperplaziy bark of adrenal glands, the arterial hypertension caused by other syndromes (Itsenko-Cushing's syndrome, malignant AG, renovaskulyarny AG, a syndrome of imaginary surplus of mineralokortikoid, etc.), nephrite with potassium loss, not diabetes, giperparateriozy, a tetaniya, a secondary aldosteronizm.
Treatment and forecast of an aldosteroma
Treatment of patients from aldosteromy consists in carrying out radical removal of a tumor together with the affected adrenal gland – adrenalektomiya. If localization of an aldosteroma is known, at surgery is applied lumbar or torako-lyumbalny accesses on the relevant party if localization is not defined - chrezbryushinny access to both adrenal glands is used.
In the preoperative period (within 7-10 days) appoint a diet with restriction of content of sodium, potassium administration of drugs (potassium chloride) and antagonists of an aldosteron - a spironolakton. For prevention of development of sharp insufficiency of bark of adrenal glands owing to surgical intervention concerning an aldosteroma therapy is shown by glucocorticoids (a cortisone, a hydrocortisone). After operation control of level of electrolytes and indicators of the ECG is necessary.
Removal of an aldosteroma in 50–70% of cases contributes to normalization or a considerable lowering of arterial pressure, in case of preservation of moderate hypertensia the correcting conservative therapy is carried out. At a good-quality aldosteroma and lack of irreversible changes from kidneys the forecast favorable. Malignant aldosteroma have an adverse current and the forecast.