Background: Adrenocortical cancer (ACC) is a rare aggressive and rapidly metastatic disease. Early diagnosis of the disease and its metastatic stage are important for the choice of treatment strategy. Evaluation of urine steroid profiles (USP) by gas chromatography-mass spectrometry (GC-MS) is a highly sensitive and specific biomarker instrument that allows for differentiation between benign and malignant tumor and obvious prospects for the diagnosis in patients with adrenal incidentalomas. In our previous study we have found no difference in urine excretion of tetrahydro-11-deoxycortisole (THS), 5-ene-pregnenes and 3,16,20-pregnenetriol/3,16,20-pregnenetriol ratio (3,16,20-dP3/3,16,20-dP3) in patients with metastatic ACC in early postoperative period, compared to pre-operative parameters. We did not account for the disease stage and primary tumor size in that study in ACC patients. Aim: To identify specific characteristics of urine steroid metabolome by GC-MS in ACC IIV stages patients before surgery in order to detect early signs of metastases and the relationship between adrenal steroidogenesis abnormalities and disease stages. Materials and methods: We performed a retrospective analysis of the data from the study of USP in 59 ACC stage I-IV patients with L. M. Weiss score 3, according to pathological examination of the surgical samples. The Cushing syndrome was diagnosed by immunochemistry assay in 28 (47.6%) of ACC patients. Tumor staging was done according to ENSAT based on the results of imaging and postoperative histological reports. ENSAT I was diagnosed in 8 patients, ENSAT II in 26, ENSAT III in 14, ENSAT IV in 11 ACC patients. The control group included 28 healthy donors. USP was assessed by GC-MS before surgery with a gas chromatography-mass-spectrometer Shimadzu GCMS-ТQ8050. Results: The first variant of urinary steroid metabolome abnormalities with increased excretion of dehydroepiandrosterone (DHEA) and THS was found in 10 (90.9%) of ENSAT IV ACC patients and in 20 (50%) of ENSAT II + III patients. The fourth USP variant was characterized by no difference in androgen and THS urinary excretion from that in healthy individuals and was found in ACC ENSAT I patients. Only in ACC ENSAT I patients, there was an increase of pregnanediol (P2) urinary excretion and of the P2/pregnanetriol (P3) ratio, compared to those in healthy donors. ROC-analysis demonstrated that ТНS 867 mcg/24 hours, 3,16,20-dP3 300 mcg/24 hours and 3,16,20-dP3/3,16,20-dP3 1.6 cut-offs had a sensitivity and specificity of 100% for preoperative identification of ENSAT IV ACC patients before surgery and for early diagnosis of ACC metastases. There were positive correlations between 16-oxo-androstenediol, THS, and progestogens, as well as a negative correlation between 3,16,20-dP3/3,16,20-dP3 ratio and the disease stage. Conclusion: Urinary excretion of THS, DHEA and its metabolites, P2, 5-ene-pregnenes, and 3,16,20-dP3/3,16,20-dP3 ratio determined by GC-MS are important biochemical markers of ACC stages and can be used as ACC metastases prognostic markers.

Aim to determine the optimal tactics for the examination and treatment of patients with macronadular bilateral adrenal hyperplasia. Material and methods. The study included 11 patients with macronodular bilateral adrenal hyperplasia (main group). To compare biochemical parameters, the results of 26 healthy people were studied (control group). Results. The patients with macronodular bilateral adrenal hyperplasia were characterized by deficiency of 11-hydroxylase, 21-hydroxylase, 11-hydroxysteroid dehydrogenase type 2 and increased activity of 5-reductase compared with the control group. The optimal treatment for these patients is unilateral adrenalectomy. A comparative selective blood sampling from the central veins of the adrenals is necessary to select the side of intervention in patients with bilateral macronodular adrenal hyperplasia with ACTH-independent Cushing's syndrome. Conclusion. The first step in the treatment of patients with bilateral macronodular adrenal hyperplasia with ACTH-independent Cushing's syndrome is unilateral adrenalectomy, which reduces the risk of developing adrenal insufficiency and subsequent hormone replacement therapy.

Introduction: Pheochromocytoma is one of representative of neuroendocrine tumors. According this fact, pheochromocytoma remains to evaluation for multiply endocrine neoplasia, especially if disease is bilateral. In this case, it is necessary to additionally carry out a genetic screening test. The aim of the study is to Improve diagnosis and treatment outcomes for patients with MEN syndrome. Materials and methods: The authors present a clinical case of a 37-year-old man diagnosed and treated for a bilateral pheochromocytoma. Results: The patient was diagnosed medullary thyroid cancer after successful bilateral adrenalectomy. The patient underwent thyroidectomy. A genetic research showed the T1900 mutation in the codon of 634 RET gene, which confirmed the presence of the patient MEN 2 syndrome. According to the high concordance of the mutated gene, patient's son was carried out to genetic examinations, which confirmed the presence of this mutation. The boy underwent thyroidectomy opportunely. Discussion: Due to primary oncological observation if the patient has pheochromacytoma, it is necessary to exclude MAN 2 syndrome both in the patient himself and his relatives. The safest and informative method is genetic research. Conclusion: presently a comprehensive examination of both the patient himself and his relatives is necessary for timely diagnosis and correct management of treatment patients with MEN 2 syndrome.

Laparoscopic adrenalectomy is the «gold standard» in adrenal neoplasm surgery. However, in the case of bilateral lesions or lesions of the only adrenal gland, it leads to the need for lifelong hormone replacement therapy. Thus, organ-preserving operations are becoming more and more in demand today. The article presents the results of laparoscopic and laparotomic resection of the adrenal gland in 32 patients with various benign adrenal neoplasms. The possibilities of organ-preserving operations on the adrenal gland were determined. Reflected approaches in the choice of partial resection of the adrenal gland.

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