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A clinical case of a man 66 y.o. who was diagnosed with hormone-inactive pituitary macroadenoma complicated by corneal erosion and partial atrophy of the optic nerve of the left eye due to exophthalmos. The increase in prolactin level was regarded due to a «stalk-effect». The patient underwent a transnasal pituitary adenomectomy with subsequent regression of symptoms. After 4 years, against the background of a new coronavirus infection, increasing general weakness, headaches, a crisis increase in blood pressure and tachycardia attacks appeared. Computed tomography (CT) accidentally revealed an adrenal incidentaloma, in laboratory tests — hypercortisolism, elevated ACTH levels, hypokalemia, hyperglycemia, increased levels of metanephrine and normetanephrine. The patient developed acute steroid psychosis, after which an adrenalectomy with a tumor was performed, a pheochromocytoma was histologically confirmed. After surgery, there was a regression of symptoms, the development of adrenal insufficiency with reduced levels of ACTH and cortisol. Upon further examination, a polynodose euthyroid goiter was established, the biopsy of the nodes — Hashimoto’s thyroiditis (Bethesda II). Meanwhile, primary hyperparathyroidism was detected. According to ultrasound, scintigraphy with Ts99m-Technetril and CT revealed an increase of left parathyroid gland. A bilateral revision of the neck, removal of the right upper and left upper parathyroid adenomas were performed. In the postoperative period, the levels of calcium and parathyroid hormone were normalized. Given the presence of a combination of multiple tumors of the endocrine system (primary hyperparathyroidism, corticotropin-producing pheochromocytoma, hormone-inactive pituitary macroadenoma, polynodose euthyroid goiter), the MEN1 syndrome was clinically established. The study of 2 and 10 exons of the MEN1 gene revealed no mutations, which does not exclude the presence of a hereditary syndrome. The patient continues observation. In the available literature in Russian and English languages the case of ACTH pheochromocytoma as part of the MEN type 1 syndrome have not been found. Therefore, we consider the presented case to be the first one.
A clinical case of a man 66 y.o. who was diagnosed with hormone-inactive pituitary macroadenoma complicated by corneal erosion and partial atrophy of the optic nerve of the left eye due to exophthalmos. The increase in prolactin level was regarded due to a «stalk-effect». The patient underwent a transnasal pituitary adenomectomy with subsequent regression of symptoms. After 4 years, against the background of a new coronavirus infection, increasing general weakness, headaches, a crisis increase in blood pressure and tachycardia attacks appeared. Computed tomography (CT) accidentally revealed an adrenal incidentaloma, in laboratory tests — hypercortisolism, elevated ACTH levels, hypokalemia, hyperglycemia, increased levels of metanephrine and normetanephrine. The patient developed acute steroid psychosis, after which an adrenalectomy with a tumor was performed, a pheochromocytoma was histologically confirmed. After surgery, there was a regression of symptoms, the development of adrenal insufficiency with reduced levels of ACTH and cortisol. Upon further examination, a polynodose euthyroid goiter was established, the biopsy of the nodes — Hashimoto’s thyroiditis (Bethesda II). Meanwhile, primary hyperparathyroidism was detected. According to ultrasound, scintigraphy with Ts99m-Technetril and CT revealed an increase of left parathyroid gland. A bilateral revision of the neck, removal of the right upper and left upper parathyroid adenomas were performed. In the postoperative period, the levels of calcium and parathyroid hormone were normalized. Given the presence of a combination of multiple tumors of the endocrine system (primary hyperparathyroidism, corticotropin-producing pheochromocytoma, hormone-inactive pituitary macroadenoma, polynodose euthyroid goiter), the MEN1 syndrome was clinically established. The study of 2 and 10 exons of the MEN1 gene revealed no mutations, which does not exclude the presence of a hereditary syndrome. The patient continues observation. In the available literature in Russian and English languages the case of ACTH pheochromocytoma as part of the MEN type 1 syndrome have not been found. Therefore, we consider the presented case to be the first one.
BACKGROUND: Adrenalectomy is the only possible method of radical treatment of adrenocortical cancer, and surgical approach (laparotomy or laparoscopy) remains a subject of debate. AIM: To evaluate one-year mortality in patients with adrenocortical cancer and determine its relationship with age, stage of the disease, tumor characteristics according to computed tomography results, and surgical approach. MATERIAL AND METHODS: In the Perm Regional Clinical Hospital, over the past 7 years, 107 patients have been operated on for adrenal tumors, of which 15 (14.01%) people were diagnosed with adrenocortical cancer. Adrenalectomy was performed by open (11) and laparoscopic (4) approaches. The results were assessed by the number of local relapses of the disease and life expectancy. Statistical analysis was carried out using standard application programs Statistica for Windows 10.0. Nonparametric methods of descriptive statistics were used. To determine the relationship between individual pairs of characteristics and the degree of its severity, the Spearman correlation coefficient (r) was calculated. RESULTS: Stage I of the disease according to the ENSAT classification was found in 1 case, II — in 7, III — in 5, IV — in 2 cases. The size of tumors removed laparoscopically was significantly smaller (p=0.042) compared to those operated on with an open approach. One-year survival rate was 80%. Surgical approach and combined operations did not affect one-year mortality (r=0.277; p=0.298 and r=–0.462; p=0.071). A relationship was found between lethal outcomes and age (r=–0.539; p=0.030), and the rate of washout of the contrast agent from the tumor in the delayed phase when performing computed tomography (r=–0.845; p=0.034). In case of lethal outcomes, the rate of washout of the contrast agent was the lowest. The life expectancy of patients depended on the stage of the disease (r=–0.590; p=0.016). CONCLUSION: One-year mortality in patients with adrenocortical cancer was 20% and had a moderate positive correlation with age, stage of the disease and a strong correlation with tumor characteristics based on bolus-enhanced computed tomography, but had no relationship with the chosen surgical approach.
BACKGROUND: Reports of the synchronous glucocorticoid-mineralocorticoid activity in patients with Cushing’s syndrome and primary hyperaldosteronism have been found in the literature for fifty years, but there are few evidence-based studies on this issue. AIM: To study mineralocorticoid and glucocorticoid secretion in patients with unilateral, bilateral tumours and bilateral hyperplasia of the adrenal cortex exploring urine and blood steroid metabolomes by chromatography methods. MATERIALS AND METHODS: 114 patients with corticotrophin-independent Cushing’s syndrome, autonomous cortisol secretion and primary hyperaldosteronism were examined. The state of the pituitary-adrenal cortex system was assessed by classical methods of immunochemical analysis and functional tests. The corticosteroid metabolomics was studied in biological fluids using high-performance liquid chromatography and gas chromatography-mass spectrometry. In accordance with functional activities and type of hyperplasia, all the patients were divided into 8 subgroups: Cushing’s syndrome and corticosteroma (n = 19), Cushing’s syndrome and bilateral adenomas (n = 9), Cushing’s syndrome and bilateral macronodular hyperplasia (n = 8); autonomus cortisol secretion and unilateral adrenal adenoma (n = 19), autonomus cortisol secretion and bilateral adenomas (n = 14) and autonomus cortisol secretion with bilateral macronodular hyperplasia (n = 11); primary hyperaldosteronism with unilateral aldosteroneproducing adrenal adenoma (n = 15) and bilateral hyperplasia (n = 19). The group of healthy subjects was a control group (n = 22). Family hyperaldosteronism and adrenocortical cancer were excluded. Patients’ urinary corticosteroid excretion and blood steroid levels were compared using the nonparametric Mann–Whitney test. RESULTS: Chromatographic methods have shown the presence of mineralocorticoid and glucocorticoid co-secretion in the patients with primary hyperaldosteronism: increased urine excretion of free cortisol 51 (27–90) ng/ml (p = 0.001) and its metabolite tetrahydrocortisol — 850 (720–994) μg/24 h (p = 0.0002) in the patients with Conn syndrome. As well as free cortisol 35 (32–72) µg/24 h (p = 0.002), tetrahydrocortisol — 1036 (490–1482) µg/24 h (p = 0.0049), 5α-tetrahydrocortisol — 1194 (411–1873) µg/24 h (p = 0.0048), 5α-tetrahydrocorticosterone — 339 (172–356) µg/24 h (p = 0.0008) in the patients with bilateral adrenal hyperplasia and hyperaldosteronism in comparison with the healthy persons. Glucocorticoid-mineralcorticoid activity was found among the patients with autonomous cortisol secretion and bilateral adrenal tumours: by increased levels of 18-hydroxycorticosterone in blood — 2.7 (1.3–3.5) ng/ml (p = 0.002). The patients with autonomous cortisol secretion and a single tumour had an increase of 18-hydroxycorticosterone in urine — 35 (33–55) µg/24 h (p = 0.0048) in comparison with the healthy subjects. The group of patients with corticotrophin-independent Cushing’s syndrome and unilateral corticosteroma was distinguished from the control group by high level of 18-hydroxycorticosterone in blood — 2.4 (1.0–4.3) ng/ml (p = 0.001) and urine — 42 (30–123) µg/24 h (p = 0.003). CONCLUSIONS: Mixed glucocorticoid and mineralocorticoid activity of adrenal cortical tumor cells was revealed among the patients with Cushing’s syndrome, with autonomous cortisol secretion and primary hyperaldosteronism using chromatographic methods.
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