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Endogenous hypercorticism (EH) is a severe symptom complex caused by hypercortisolemia; according to the etiology, ACTH-dependent and ACTH-independent variants are distinguished, which, according to the literature, occur in 70-80% and 20-30% of cases, respectively. A rare cause of ACTH-dependent endogenous hypercorticism is ACTH-ectopic syndrome (ACTH-ES) (about 15-20% of cases). ACTH-ES is a syndrome of adrenocorticotropic hormone (ACTH) hyperproduction by neuroendocrine tumors of extrahypophyseal origin. Various tumors can secrete ACTH: bronchopulmonary carcinoid, small cell lung cancer, less frequently, thymus carcinoid, islet cell tumors and pancreatic carcinoid, medullary thyroid cancer, carcinoid tumors of the intestine, ovaries, as well as pheochromocytoma (PCC).This publication presents a clinical case of rarely detected paraneoplastic ACTH production by pheochromocytoma. The patient had clinical manifestations of hypercorticism, therefore, she applied to the Russian National Research Center of Endocrinology of the Ministry of Health of Russia. During the examination Cushing's syndrome (CS) was confirmed, multispiral computed tomography (MSCT) of the abdominal cavity revealed a voluminous formation of the left adrenal gland. Additional examination recorded a multiple increase in urinary catecholamine levels. Subsequently, the patient underwent left-sided adrenalectomy. The diagnosis of pheochromocytoma was confirmed morphologically, immunohistochemical study demonstrated intensive expression of chromogranin A and ACTH by tumor cells.

Combined staged ultrasonic examination of 53 patients with surgical diseases of the adrenals (Icenko--Cushing's disease and hormonally active tumors) included preoperative transcutaneous ultrasonic examination, ultrasound-monitored spot fine needle aspiration biopsy, intraoperative and postoperative ultrasonic examinations. Transcutaneous ultrasonic examination was found to be little informative in Icenko--Cushing's disease, whereas in adrenal tumors it was virtually as sensitive as computer-aided tomography. Ultrasound-monitored spot fine needle aspiration biopsy proved to be a highly informative safe method, which helps morphologically diagnose the disease before surgery. Intraoperative ultrasonic examination helped reduce the surgical trauma in all the cases, and in patients with Icenko--Cushing's disease it gave an idea of the type of morphologic changes in the removed adrenal. Ultrasonic monitoring of tissue status at the site of intervention helped timely detect the postoperative complications.

The authors analyze published data on clinical variants and hormonal parameters in patients with this syndrome and present their own observations of children with this condition. Besides early sexual maturation, they describe the clinical picture of Cushing's syndrome, thyroid nodular hyperplasia. The said disorders do not depend on the tropic effect of the hypothalamohypophyseal system. A hypothesis is put forward about primary activation of adenylate cyclase system in the origin of the disease.

The paper is devoted to surgical treatment of patients with Itsenko-Cushing disease. Altogether 16 patients after adrenalectomy with subsequent autotransplantation of the gland on a vascular pedicle were investigated. The purpose was to study a morphological picture of the gland in ischemia and the determination of time required for blood flow recovery in the gland. Light and electron microscopy, radiological and clinical methods were employed. Emerging of the removed gland before autotransplantation into a nutrient medium for 30 min. (before blood flow recovery in a graft) was shown to damage neither secretory cells nor the components of the microcirculatory bed. The extracorporal condition of the gland for 1 h was accompanied by an increase in autolytic processes, especially in the medullary substance and deep layers of reticulated cortical zone, but most of the adrenocorticocytes preserved their viability and structural features of steroid activity, which became an indication for effective autotransplantation of the adrenal gland on a vascular pedicle in the treatment of Itsenko-Cushing disease.

Proceeding from a study of the results of the investigation of 152 patients with Icenko-Cushing's syndrome the authors have arrived at the conclusion that hyperplasia, clear cell adenoma of monomorphous structure, dark cell and mixed adenomas of polymorphous structure, cancer of the adrenal cortex are different stages of a common pathological process. Such formation of morphological changes in the adrenal gland is one of the ways of development of Icenko-Cushing's syndrome. To confirm the proposed concept of the pathogenesis of this disease the authors provide some evidence of hyperplasia or the normal structure of nontumorous adrenocortical tissue in adenoma and cancer, of the absence of difference in a rise of the mean ACTH level in the blood in hyperplasia and different adrenocortical tumors. An increase in ACTH production in most of the patients should be regarded as one of the primary factors of the above morphological changes. The absence of suppression of 17-oxycorticosteroid excretion with urine in a test with 8 mg of dexamethasone in most of the patients with dark cell and mixed adenoma of polymorphous structure and adrenocortical cancer suggests a possibility of the development of certain autonomy of these neoplasms.

The paper is concerned with the use of a new tomographic method based on the principle of nuclear magnetic resonance (NMR-tomography) in the diagnosis of adenohypophyseal diseases. A NMR-tomograph with a resistive magnet (0.234 T) was employed. Altogether 15 patients with the most common types of hypothalamo-hypophyseal diseases including Itsenko-Cushing disease (6) of various degrees of severity, hyperprolactinemic hypogonadism (8), and one patient with acromegaly were investigated. NMR-tomography was shown to possess a high informative value permitting assessment of the presence of adenohypophyseal pathology without irradiation of a patient. NMR-tomography combined with routine methods of examination of patients with hypothalamohypophyseal pathology (roentgenography, computerized tomography, hormonal investigation) made it possible to improve considerably early diagnosis of pituitary adenomas and to specify the volume and type of tumor growth.

The authors present a description of two observations over patients with aseptic necrosis of the heads of the femur in Itsenko-Cushing's disease. A short literature survey is given, and an opinion is put forward that not only osteoporosis, but also other factors (thrombosis, vasculitis, and others) underly the lesion pathogenesis.

The frequency and nature of the main signs of Icenko-Cushing's syndrome were studied in 132 patients divided into 4 groups with relation to morphological changes in the adrenals. It was established that the peculiarities of morphological structure of the adrenals were not reflected in a clinical picture of disease. A more frequent development of some clinical manifestations of Icenko-Cushing's syndrome was noted in the group of patients with a significantly higher mean level of cortisol in the blood plasma. The test with 8 mg of dexamethasone for suppression of 17-OCS excreted with urine was not strictly specific for any stage of morphological changes in the adrenal cortex.