Symptoms of the syndrome of ectopic production of ACTH

Symptoms of the syndrome of ectopic production ACTH is a different degree of hypercorticism. In the case of rapid progression of the tumor process and high production of hormones, the adrenal cortex develops a typical Itenko-Cushing syndrome. In patients, excessive deposition of subcutaneous fat in the face, neck, trunk, abdomen is noted. The face acquires the form of a "full moon". The limbs are thin, the skin becomes dry, acquires a purplish-cyanotic color. Appear red-violet strips of "stretching" on the skin of the abdomen, thighs, inner surfaces of the shoulders. There is a general, and in places of friction, hyperpigmentation of the skin. On the skin of the face, chest, back appears hypertrichosis. There is a tendency to furunculosis and development of erysipelas. The arterial pressure is increased. The skeleton has been osteoporotically changed, with a severe course there are fractures of the ribs and vertebrae. Steroid diabetes is characterized by insulin resistance. Hypokalemia of varying degrees depends on the degree of hypercortisy. The development of his symptoms depends on the biological activity and the amount of hormones secreted by the tumor and secreted by the cortex of the adrenals of cortisol, corticosterone, aldosterone and androgens.

One of the characteristic and persistent symptoms of the syndrome of ectopic production of ACTH is a progressive muscle weakness. It is expressed by fast fatigue, pronounced fatigue. To a greater degree this is observed in the lower limbs. The muscles become flabby and soft. Patients can not get up from the chair or climb the stairs without help. Often, physical asthenia in these patients is accompanied by mental disorders.

These symptoms are caused by hypokalemia, which is a consequence of increased potassium excretion under the influence of excessive production of cortisol. The potassium content in the plasma is usually 3 mmol / l. Its excretion in the syndrome of ectopic production of ACTH sometimes reaches a large size and leads to the development of so-called potassium diabetes. At the same time, the level of potassium in the muscles and heart decreases, which is expressed by characteristic changes on the ECG, the alkaline reserve of blood and the level of bicarbonates rises. As a result of the removal of large amounts of this substance from the cells and replacement of sodium and hydrogen ions with it by ions of sodium and hydrogen, hypocalcemic alkalosis develops, which is combined with compensatory reduction of chlorine, in most patients hypochloraemia is observed. Increased blood volume contributes to the development of hypertension in patients.

Hyperpigmentation of the skin and mucous membranes is a characteristic manifestation of the syndrome of ectopic AKTH production. The shades of pigmentation may be different (smoky, chocolate, brown, almost black with a blue tint). Sometimes strengthening the color of the skin for a long time may be the only sign of the manifestation of an ectopic tumor. In some patients, hyperpigmentation develops simultaneously with symptoms of hypercorticism.

The development of enhanced pigmentation in the skin depends on the secretion of the tumor-ectopic ACTH. And its properties may differ from ACTH of hypophyseal origin. Therefore, the hormone differently affects the coloration of the skin and stimulation of the adrenal glands. Melanoderma, which develops in the syndrome of ectopic secretion of adrenocorticotropic hormone, can be compared with hyperpigmentation of the skin in patients with pituitary tumor, Nelson's syndrome and Addison's disease.

For most patients, the clinical picture of hypercorticism is uncharacteristic. They do not have a kind of obesity, on the contrary, cachexia often develops. The predominant symptoms are progressive muscle weakness, hyperpigmentation of the skin and mucous membranes, hypokalemic alkalosis, hypertension, tolerance to carbohydrates, emotional lability.

In some patients, ACTH and CRF were detected in tumors, but no clinical manifestations of their presence were observed. The reason for this is either a small activity of the compounds secreted by the tumor or a lack of time for the development of symptoms of hypercorticism. Thus, the clinical manifestations in patients with the syndrome of ectopic ACTH can be typical of the Itenko-Cushing syndrome or partial.

The symptoms of the disease can develop rapidly (for several months) or slowly (over several years). In addition to the changes inherent in hypercorticism, in patients with the syndrome of ectopic secretion of ACTH, signs characteristic of the tumor process appear. Often they have an intoxication, manifestations of metastasis in various organs, symptoms of compression of the vascular-plexus plexuses. Clinical manifestations of the syndrome of ectopic production of ACTH depend not only on hypercorticism, but also on other hormones that can secrete a tumor.

Patients with an ovate cell tumor of the bronchi are described who, in addition to ACTH production, have ADH secretion. The joint action of these hormones masked the development of hypokalemia. It is believed that the asymptomatic increase in ADH secretion occurs quite often.

Very rare cases of a combination of ectopic products of ACTH and growth hormone. A 37-year-old patient with clinical manifestations of acromegaly, hypercortisy is described; Malignant bronchial carcinoid contained ACTH and STH

There are data on an 18-year-old patient with gigantism, Itenko-Cushing syndrome. After autopsy, ACTH and STH were isolated from carcinoid metastases in the liver. In addition, somatotropinoma was found.

Tumor secretion was reported along with ACTH vasopressin, oxytocin, and neurofizin. The authors rely on the determination of osmolality of serum and urine. The presence of vasopressin is detected by reducing the ability of patients to respond to water stress.