[Surgical treatment of pheochromocytoma].

This review article contains a summary of modern aspects of preoperative preparation, surgical treatment, and follow-up of patients with adrenal pheochromocytomas. The main component of preoperative preparation is the use of alpha-blockers. The need to prescribe them to all patients is increasingly disputed, especially for patients without severe hypertension. An increasing number of publications demonstrate positive results of treatment without the use of alpha-blockers, advocating an individual approach and the use of the drug according to certain indications. Minimally invasive endoscopic techniques of adrenalectomy have become widespread in surgical treatment. They are represented by laparoscopic and retroperitonescopic technic, including using their single-port modifications. The earliest possible intersection of the central vein in the past was considered the most important aspect of adrenalectomy for pheochromocytoma, currently, due to the development of surgical techniques and anesthesiological manuals, this has ceased to be a mandatory rule of successful surgery. Despite the significant influence of the intersection of this vessel on intraoperative hemodynamics, surgical tactics with its later intersection have their own justifications and do not lead to a deterioration in treatment results. The standard volume of surgical intervention for pheochromocytomas is total adrenalectomy, however, in the presence of hereditary syndromes, such as multiple endocrine neoplasia type 2 syndrome, neurofibomatosis type 1, von Hippel-Lindau syndrome, it is possible to perform cortical-sparing adrenalectomy.

[Corticotropin-producing pheochromocytoma in multiple endocrine neoplasia type 1].

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-effect¼. 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.

[Diagnostic value of ct in examination of patients with adrenal cancer].

BACKGROUND: In most cases adrenal tumours are detected by accident while performing medical imaging tests for other diseases. These findings are treated as adrenal incidentaloma. Prevalence of incidentalomas detected on CT scans is up to 4%. According to different authors, 4-12% of all adrenal tumours are adrenocortical carcinomas. As for today, the most significant medical imaging technique is CT scan with bolus IV injection of contrast agent and assessment of tumour's density. The analysis of the results of CT imaging in 67 patients with ACC was carried out according to a single protocol. The main signs characteristic of this disease are described. It is very important to evaluate typical signs of ACC on CT scans for risk assessment of ACC before surgical treatment. If malignant tumour is suspected during preoperative examination, it is extremely important to choose the right surgical treatment strategy. AIM: To evaluate the significance of CT as the main method of preoperative diagnosis in patients with malignant tumors of the adrenal cortex. Studying CT semiotics of adrenocortical cancer in a large group of patients using a single standard imaging protocol. Find the main radiological symptoms characteristic of adrenocortical cancerMATERIALS AND METHODS: Here are the results of retrospective study of CT scans performed on 67 patients with adrenocortical carcinoma who received treatment in the Department of Endocrine Surgery of Saint-Petersburg State University N.I. Pirogov Clinic of High Medical Technologies during 2012-2020. The diagnostic significance of CT in patients with ACC was assessed. RESULTS: The most common features of ACC: tumour heterogeneity (84.3%), tumour's size 3-9 cm (75%), signs of invasion into surrounding structures (10%), pre-contrast density above +30 HU (75%), absolute contrast washout less than 60% (68.8%), relative contrast washout less than 40% (64.6%)CONCLUSION: CT scan with IV contrast was not able to show any definitive pathognomonic signs of ACC. Nevertheless, CT scan should be performed in all patients with suspected (or confirmed using other medical imaging technique) adrenal tumour according to standard protocol. Bolus injection of contrast agent should be performed in all patients with tumour's pre-contrast density above +5 HU.

[Clinical and laboratory features of hereditary pheochromocytoma and paraganglioma].

The widespread introduction of genetic testing in recent years has made it possible to determine that more than a third of cases of pheochromocytomas and paragangliomas (PPPGs) are caused by germline mutations. Despite the variety of catecholamine-producing tumors manifestations, there is a sufficient number of clinical and laboratory landmarks that suggest a hereditary genesis of the disease and even a specific syndrome. These include a family history, age of patient, presence of concomitant conditions, and symptoms of the disease. Considering that each of the mutations is associated with certain diseases that often determine tactics of treatment and examination of a patient, e.g. high risk of various malignancies. Awareness of the practitioner on the peculiarities of the course of family forms of PPPGs will allow improving the tactics of managing these patients.The article provides up-to-date information on the prevalence of hereditary PPPGs. The modern views on the pathogenesis of the disease induced by different mutations are presented. The main hereditary syndromes associated with PPPGs are described, including multiple endocrine neoplasia syndrome type 2A and 2B, type 1 neurofibromatosis, von Hippel-Lindau syndrome, hereditary paraganglioma syndrome, as well as clinical and laboratory features of the tumor in these conditions. The main positions on the necessity of genetic screening in patients with PPPGs are given.