Первичный гиперпаратиреоз занимает третье место по распространенности среди эндокринных заболеваний после сахарного диабета и патологии щитовидной железы, его частота во взрослой популяции составляет в среднем 1-2%. Наиболее часто при первичном гиперпаратиреозе выявляется солитарная аденома около-щитовидной железы (ОЩЖ) -80-85% случаев, реже гиперплазия и множественное поражение (до 15%), рак ОЩЖ встречается примерно в 1% наблюдений. Среди аденом ОЩЖ отдельно выделяют группу клинически агрессивных аденом, ассоциированных с более тяжелым течением заболевания, выраженной гиперкальциемией и неуточненным злокачественным потенциалом. В настоящее время нет четких критериев, которые помогли бы на дооперационном этапе дифференцировать атипическую аденому от карциномы и от "классической" аденомы. На сегодняшний день не существует критериев, позволивших бы на дооперационном этапе отличить атипическую аденому от типичной или рака ОЩЖ. Мы представляем случай 61-летней пациентки с клинически агрессивным течением первичного гиперпаратиреоза и выраженными метаболическими нарушениями структуры костной ткани очагового и диффузно-очагового характера вследствие атипической аденомы ОЩЖ. Ключевые слова: первичный гиперпаратиреоз, атипическая аденома околощитовидной железы, фиброзно-кистозный остеит, рак околощитовидных желез, остеосцинтиграфия, радионуклидная диаг ностика.Primary hyperparathyroidism is common clinical endocrine disorder with a prevalence between 1-2%. Solitary parathyroid adenomas account from 80 to 85% of cases of PHPT, hyperplasia and multiple adenomas is up to 15%, parathyroid carcinoma is a very rare cause of PHPT, accounting for about a 1% of cases. Clinically aggressive and atypical adenomas should be separately noted because of severe clinical course and life-threatening hypercalcemia, high morbidity and mortality, unknown malignant potential. No definite criteria are considered to be present to distinguish preoperatively atypical adenoma from parathyroid typical adenoma or carcinoma. The clinical course of the disease remains the only tool that allows to suspect an aggressive tumor on the preoperative stage. We present the clinical case of a 61-year-old female patient with a clinically "aggressive" course of PHPT and severe metabolic disturbances of bone tissue due to the atypical adenoma of the parathyroid gland.Key words: primary hyperparathyroidism, atypical parathyroid adenoma, cancer of the parathyroid glands, osteitis fibrosa cystica, osteoscintigraphy, radionuclide diagnostics.The article can used under the CC BY-NC-ND 4.0 license. Статья может быть использована на условиях международной лицензии CC BY-NC-ND 4.0.

Central Military Clinical Hospital named after P.V. Mandryko, Moscow, Russian FederationФеохромоцитомы и параганглиомы (ФХ/ПГ) -редкие катехоламин-секретирующие нейроэндокринные опухоли, почти в 40% случаев имеющие наследственную природу. Заболеваемость колеблется от 2 до 8 случаев на 1 млн человек в год, с пиком заболеваемости в 30-50 лет. Согласно последней классификации, хромаффинные опухоли отнесены к злокачественным новообразованиям. Частота метастазирования ФХ -10%, ПГ -25%. Клинические проявления ФХ и ПГ обусловлены избытком катехоламинов. Известно более 20 наследуемых генов, мутации в которых провоцируют развитие ФХ/ПГ. С точки зрения молекулярной клеточной патофизиологии известный на сегодня пул мутаций можно разделить на два кластера: первый (SDHх, SDHAF2 -фактор сборки SDH, FH, MDH2) нарушает функционирование цикла Кребса и энергетической транспортной цепи митохондрий, второй (RET, NF1, TMEM127, MAX) -мутации генов рецепторов трансмембранных белков-протеинкиназ (тирозинкиназ), активирующие внутриклеточные сигнальные пути (PI3K-AKT-mTOR и MYC), ответственные за клеточный рост, регуляцию роста и дифференцировку клеток. В итоге происходят стабилизация HIF-транскрипционных факторов (оксидативный стресс), изменение метилирования ДНК, приводящие в итоге к глубоким нарушениям экспрессии генов и опухолевой трансформации клетки. Выделяют три основных секреторно-биохимических фенотипа ФХ/ПГ: норадренергический, адренергический и допаминергический. В зависимости от типа секреции опухоли, возраста пациента и семейного анамнеза назначаются комплементарные генетические исследования и методы молекулярной визуализации. В клинической практике биохимический фенотип опухоли, стадия, семейный анамнез и особенно генетический "паспорт" опухоли позволяют подобрать оптимальный алгоритм молекулярной визуализации (ОФЭКТ/ПЭТ) в целях персонализации тактики лечения и клинического прогноза.Ключевые слова : хромаффинные опухоли, феохромоцитома, параганглиома, радионуклидная диагностика, молекулярная визуализация, генетика, эндокринология, онкология, радиология, онкоэндокринология. Pheochromocytomas and paragangliomas (PPGLs) are rare catecholamine-secreting neuroendocrine tumours, up to 40% of which occur in the setting of a hereditary syndrome. The incidence is 2 to 8 per million persons per year. The peak incidence occurs in the third to fifth decades of life. According to the most recent classification, chromaffin tumours refer to malignant neoplasms. The incidence of metastasis in pheochromocytomas is 10%; in paragangliomas it is 25%. Clinical manifestations of PPGLs are caused by the excess of catecholamines. More than 20 hereditary gene mutations are known to result in PPGLs development. According to the molecular and cellular pathophysiology, all currently known mutations can be divided intoThe article can used under the CC BY-NC-ND 4.0 license. Статья может быть использована на условиях международной лицензии CC BY-NC-ND 4.0.

Phosphaturic mesenchymal tumor can cause osteomalacia due to excessive secretion of fibroblast growth factor 23 (FGF23), which disrupts the metabolism of phosphate and vitamin D. These tumors are predominantly benign, but less than 5% of them are malignant forms. This article presents the first clinical case in the Russian Federation of a 69-year-old patient with severe hypophosphatemia due to metastatic prostate cancer. Increased secretion of FGF23 are described in the androgen-resistent prostate cancer, which led to pronounced disorders of mineral metabolism, accompanied by a clinical symptom of weakness, pain in the bones, immobilization of the patient. The condition was regarded as worsening against the background of the progression of the disease. However, symptomatic therapy aimed at increasing the level of phosphate significantly improved the patient’s general condition. The medical community should be aware of the possibility of developing hypophosphatemia in patients with weakness and bone pain, which are not always associated with the progression of metastatic prostate cancer.

Introduction. Primary hyperparathyroidism is one of the most common diseases of the endocrine system, after diabetes mellitus and thyroid pathologies. Early diagnosis and treatment of primary hyperparathyroidism allow avoiding severe damage to the bones, kidneys, other organs, thereby reducing the incidence of disability and improving the patients quality of life. The only radical treatment for primary hyperparathyroidism is the surgical removal of the pathologically altered, hyperfunctioning parathyroid glands.The study objective – to increase the efficiency of preoperative topical diagnosis and intraoperative navigation of parathyroid glands.Materials and methods. 200 patients with laboratory-verified primary hyperparathyroidism, who underwent preoperative topical diagnostics (ultrasound, planar scintigraphy and single-photon emission computed tomography, combined with computed tomography (SPECT / CT), in some cases supplemented with contrast enhanced CT with / or fine needle aspiration biopsy with flushing from a needle on for parathyroid hormone) and received surgical treatment, in period from 2017 to 2020. A single-stage, open-label comparative study was carried out, including clinical, laboratory and instrumental data of patients. The follow-up period after surgery for primary hyperparathyroidism was at least 6 months.Results. From 200 included patients, surgical treatment in the amount of minimally invasive parathyroidectomy was performed in 189 patients. As a result of the analysis of the diagnostic accuracy, for a combination of ultrasound and SPECT/CT with augmented contrast enhanced CT, the overall accuracy in visualizing of parathyroid glands was 99 % (95 % confidence interval (CI): 97–100), diagnostic specificity 77 % (95 % CI: 54–100), sensitivity 100 % (95 % CI: 98–100), the predictive value of positive and negative results was 98 % (95 % CI: 97–100) and 100 % (95 % CI: 98–100) respectively.Conclusion. The results allowed us to develop an algorithm for preoperative topical diagnosis of parathyroid glands in patients with laboratory-verified primary hyperparathyroidism and indications for surgical treatmen. We recommend to perform ultrasound of the thyroid and parathyroid glands in all patients at the first stage, in the absence of evident changes in the thyroid gland, at the second stage – scintigraphy and SPECT / CT with 99mTc-MIBI; in cases with significant concomitant functional or structural pathology of the thyroid gland – contrast enhanced CT; if necessary, supplementing fine needle aspiration biopsy with flushing from a needle on for parathyroid hormone.

BACKGROUND: Insulinoma is a pancreatic neuroendocrine tumor that manifests by impaired carbohydrate metabolism with the development of hypoglycemic syndrome. The instrumental methods used at the present stage do not always make it possible to identify a tumor; moreover, the data obtained often contradict each other. Thus, the search for new possibilities of visualization of insulinoma is relevant.AIM: Evaluation of diagnostic effectiveness of scintigraphy with single-photon emission computed tomography combined with X-ray computed tomography (SPECT/CT) with 99mTc-Tectrotide for insulinoma in a Russian cohort of patients.MATERIALS AND METHODS: A single-centre (Endocrinology Research Centre of the Ministry of Health of the Russia), experimental, single-stage, controlled study. In the years 2017–2021 patients with pancreatic insulinoma (group 1) and hyperinsulinemic hypoglycemia of a different genesis (group 2) with negative or contradictory results of the 1st line imaging methods (ultrasound, magnetic resonance imaging (MRI), computed tomography (CT)) were included. All participants underwent the whole-body scintigraphy and low-dose SPECT/CT with 99mTc-Tectrotide (500–900 MBq). The studies were performed on a tomograph of the SPECT/GE Discovery NM/CT 670 using low-energy high-resolution collimators (LEHR) in the «whole body» mode.RESULTS: In the group 1 (n=21), according to the results of a pathomorphological study, the presence of 26 insulin-producing tumors was confirmed. Group 2 included 9 patients. According to the SPECT/CT with 99mTc-Tectrotide, 14 tumors were diagnosed in group 1 out of 26 insulin-producing tumors of the pancreas, and negative results were obtained in group 2 in 100% of cases. Thus, the sensitivity and specificity of the method were: 54%, 95% CI [33%; 73%] and 100%, 95% CI [68%; 100%], respectively.CONCLUSION: SPECT/CT with 99mTc-Tectrotide can detect insulinoma in 54% of cases with negative or contradictory results of 1st-line imaging methods (ultrasound, MRI, CT). This study can be effectively used as an alternative to SPECT/CT with 111In-octreotide, as a 2nd-line method for topical search for an insulin-producing pancreatic tumor.