[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.

Quality of life in patients with primary hyperparathyroidism before and after parathyroidectomy: long term single center experience.

BACKGROUND: Primary hyperparathyroidism (PHPT) is a common endocrine disorder caused by a parathyroid tumor or hyperplasia, which is often accompanied with quality of life (QoL) impairment. A parathyroidectomy (PTX) is the preferred standard treatment for PHPT patients. In this single center study we aimed to evaluate the impact of PHPT on patient's QoL and identify QoL changes at early and long-term follow-up after surgery. METHODS: All the patients underwent routine PTX with the removal of the suspected hyperparathyroid gland(s). Patients filled out generic QoL questionnaire RAND SF-36, specific questionnaire PHPQoL and specific symptom assessment questionnaire PAS upon admission to the hospital before surgery, at 3 months, 12 months and 24 months after surgery. RESULTS: A total of 92 patients with PHPT (median age was 56 years, 95.7% females) were included in the study. Before PTX patient's QoL by SF-36 scores was significantly lower as compared to healthy controls (p < 0.01). Almost 40% of patients had poor or very poor QoL. The most frequent symptoms by PAS before surgery were as follows: tiredness (97.8% of patients), weakness (94.6%), forgetfulness (94.6%), mood changes (90%), feeling "blue"/depression (88%), joint pains (83.3%), headaches (80.2%), constant irritability (77.2%), bone pains (75%), thirst (70.7%) and trouble getting out of a chair (67.4%). The half of the patients had moderate-to-severe (≥ 40 scores) tiredness, weakness, joint pains, forgetfulness, as well as mood changes. Post-operative QoL changes were analysed in the group of 72 patients. After surgery there was significant improvement in QoL by all scales of SF-36 questionnaire, excluding bodily pain, and the PHPQoL total score (GEE, p < 0.01) as compared with their values before surgery. Also severity of tiredness, mood changes, weakness and forgetfulness significantly decreased after surgery as compared to their baseline values (GEE, p < 0.05). Decreased mental component of QoL by PHPQoL (OR = 0.927, 95%CI = 0.874-0.984, p = 0.013) predicted improved QoL after surgery. CONCLUSIONS: Patients with PHPT demonstrated significantly impaired QoL in physical, psychological and social functioning as well experienced a wide profile of common PHPT symptoms. Successful PTX was accompanied with remarkable QoL improvement and decrease in subjective symptoms for at least 24 months after surgery.

[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.

[Quality of life in patients with primary hyperparathyroidism after surgery].

BACKGROUND: For a comprehensive assessment of the effect of surgery in patients with primary hyperparathyroidism (PHPT), as well as for monitoring the condition of patients after treatment, it sounds reasonable to evaluate quality of life (QoL) and symptoms in PHPT patients before and after surgery. AIM: The aim of this study was to assess changes in the QoL and symptoms in patients with PHPT after surgery. MATERIALS AND METHODS: During prospective observational study, patients filled out QoL questionnaires and evaluated the presence and severity of their symptoms prior to parathyroidectomy (PTE) and 3, 12 months after surgery. Statistical analysis included the following methods: Student's t-test or Wilcoxon's non-parametric test, the generalized estimating equations (GEE), correlation analysis, χ2 and McNemar tests. RESULTS: The study included 72 patients (mean age 52 years, 97.2% female) with symptomatic (68.1%) and asymptomatic (31.9%) PHPT. Before surgery patients with PHPT exhibited significantly decreased role functioning, physical and social well-being, and vitality. Half of PHPT patients experienced moderate-to-severe symptoms such as weakness, fatigue, loss of concentration, mood changes, as well as joint and bone pain; the association between symptoms experienced and the extent of QoL impairment before surgery was shown. Three months after PTE improvement in both physical and psychological components of QoL was shown. Positive QoL changes were demonstrated in patients with both symptomatic and asymptomatic PHPT and they preserved for 12 months after surgery. Also within 12 months after PTE significant decrease in PHPT-associated symptoms such as weakness, fatigue, loss of concentration and mood changes was found. CONCLUSION: The results obtained demonstrate efficacy of PTE from the patient's perspective and confirm the value of QoL assessment in PHPT patients in management of this patients' population both for decision making and for evaluation of benefits of surgery and the degree of recovery of patients at long term follow-up.

[Role of preoperative visualization in the choice of surgery for primary hyperparathyroidism].

BACKGROUND: Precise localization of abnormal parathyroid glands is important for a successful surgery for primary hyperparathyroidism (PHPT). While a large number of patients can be successfully treated with the focused parathyroidectomy, there is a considerable rate of the persistent PHPT mostly because of undetected multiglandular disease (MGD). AIM: The aim of the study was to evaluate the meaning of preoperative visualization data for planning the surgery for patients with PHPT. MATERIALS AND METHODS: The study was conducted at SPBU Hospital in 2017-2018. 810 patients who underwent a primary surgery for PHPT were included in the study. Preoperative imaging results were investigated and multivariative logistic regressions were calculated to assess the predictive values of preoperative data. The rate of cases with persistent disease and cases with MGD were compared between patients with different results of preoperative data. RESULTS: Age, sex, body mass index, negative results of preoperative US, MIBI and 4D CT were not independently associated with the higher risk of multiglandular disease. The larger number of performed preoperative visualization studies were associated with the higher risk of persistence. 37% cases of MGD were not identified preoperatively. There were 7 cases with previously unsuspected second adenomas found only due to bilateral neck exploration. CONCLUSION: Any combination of preoperative visualization modalities was not able to rule out the MGD reliably. Efficacy of surgical treatment was not associated with the higher number of preoperative studies. Bilateral neck exploration may decrease the rate of the persistent hyperparathyroidism improving the identification of multiglandular disease.

[Validation and testing of the Russian version of PHPQoL questionnaire for quality of life assessment in patients with primary hyperparathyroidism (PHPT)].

BACKGROUND: Quality of life (QoL) assessment before and after surgical treatment in patients with primary hyperparathyroidism (PHTP) may be useful for comprehensive evaluation of the treatment effect, as well as for monitoring of the patient' condition after surgery, including in real clinical practice. AIM: The aim of the study was to validate and test the Russian version of the PHPQoL questionnaire for assessment of the quality of life (QoL) in patients with primary hyperparathyroidism (PHPT) for further application in clinical practice and research in Russia. MATERIALS AND METHODS: The linguistic and cultural adaptation of the PHPQoL questionnaire was carried out in accordance with international guidelines. Psychometric properties of the tool, namely, its reliability, validity and sensitivity were tested in the focus group of patients with PHPTResults: In the whole, 65 patients with PHPT were involved into the study (mean age - 52.3 ± 10.5 years, 97% - -women): 67.7% patients were symptomatic, 35.4% patients had moderate or severe hypercalcemia. All the patients filled out the Russian version of PHPQoL before parathyroidectomy. One third of patients filled out the Russian version of PHPQoL twice - before surgery and 3 months after surgery. Satisfactory external and content validity of the Russian version of PHPQoL was demonstrated. Its stable structure confirmed satisfactory construct validity of the questionnaire. The ability of the tool to determine differences in severity of symptoms/problems due to PHPT before and after treatment was revealed. The positive effect of the surgery on QoL in PHPT patients was shown. CONCLUSION: The results obtained during the study confirm that the Russian version of PHPQoL is a reliable, valid and sensitive tool. Feasibility and applicability of its use in research and clinical practice in Russian endocrinology settings has been demonstrated.

[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.