ВАРИАНТЫ ТЕЧЕНИЯ СИНДРОМА ЭКТОПИЧЕСКОЙ ПРОДУКЦИИ АДРЕНОКОРТИКОТРОПНОГО ГОРМОНА ПРИ КАРЦИНОИДЕ ЛЕГКОГО Максим О.В. 1 , Фуфаев Е.Е. 1 , Шустов С.Б. 1 , Баринов О.В. 1 , Шафигуллина З.Р. 2 , Лисицын А

Objectives to reveal the clinical, morphological and hormonal characteristics of patients operated on for isolated adrenal myelolipoma and myxoid adenomas with adrenal myelolipoma. Material and methods.The study was based on the results of examination of patients operated on for adrenal myelolipoma in the period from 2000 to 2015. During the study, the hormonal activity in patients with isolated and myxoid adrenal myelolipomas was analyzed. A pathomorphological and immunohistochemical study of the removed formations of the adrenal gland was performed. The article presents rare clinical observations of patients undergoing treatment in the clinic of E.E. Eichwald, North-Western State Medical University named after I.I. Mechnikov, with accidentally detected neoplasms, which were a combination of adenoma and myelolipoma of the adrenal gland. Results.The occurrence of isolated myelolipomas and myxoid adenomas with myelolipoma among incidentalomas was 6.3% and 4.9%, respectively. The average age of patients with adrenal myelolipoma is 54.5 years. The ratio of women to men was 2:1. According to our data, four types of hormonal activity have been identified among myxoid formations of the adrenal gland. Type 1 is associated with Cushing's syndrome, type 2 is associated with Conn's syndrome, type 3 is associated with autonomic cortisol secretion, type 4 has no hormonal activity. Based on the results obtained by high-performance liquid chromatography (HPLC), 11-hydroxylase deficiency was noted, and according to the study of the urine steroid profile by gas chromatography-mass spectrometry (GCMS), the signs of type 2 11-hydroxysteroid dehydrogenase deficiency and an increase in 5-reductase activity in patients with isolated adrenal myelolipoma were obtained. Conclusion.The combination of adenoma with myelolipoma, as well as of adenoma, myelolipoma and cavernous hemangioma is a rare pathology, the diagnosis of which is based on the pathomorphological examination of the removed tumor. Myelolipoma as a part of adrenal adenoma is an incidentaloma, which can be of different sizes and is accompanied by hormonal dysfunction of the cells of the adrenal cortex. An uncertain intrascopic phenotype and hormonal activity of myxoid formations are the decisive factors in favor of surgical intervention.

The article presents the analysis of renin–angiotensin system and content precursor of aldosterone and their metabolites, investigated by methods of high-performance liquid chromatography and gas chromatography – mass-spectrometry in 49 patients with pheochromocytoma (РНЕО) and 28 patients with non-active adenoma of the adrenal cortex and 26 healthy people. In the group of patients with PHEO the increased blood levels оf aldosterone and urine excretion of 18-hydroxycorticosterone, tetrahydrocorticosterone and it 5-α form were obtained. These patients had features of hyporeninemic and hyperreninemic hyperaldosteronism. Positive correlations of urinary excretion of free normetanephrine with blood aldosterone, renin, corticosterone and 18-hydroxycorticosterone suggest the relationship between adrenal cortex and medulla.