Актуальность. Патология паращитовидных желез по частоте встречаемости находится на третьем месте среди эндокринных болезней, уступая сахарному диабету и заболеваниям щитовидной железы. На сегодняшний день в клинической практике широко применяются только два метода лечения гиперпаратиреоза: консервативный и хирургический. Однако в последние время помимо них появились способы транскутанной термодеструкции (аблации), основанные на прицельном физическом воздействии: лазерном, радиочастотном, микроволновом, ультразвуковом. Цель исследования . Настоящий обзор посвящен критическому анализу современного арсенала методов локальной термодеструкции гиперфункции паращитовидных желез при гиперпаратиреозе. Материалы и методы. В обзор включены данные рандомизированных клинических исследований за период с 2012 года по 2021 год, найденных в Google Scholar, Pubmed. Общее количество пациентов 1983 (лазерная аблация - 216 пациентов, радиочастотная аблация - 225, микроволновая аблация - 1467, аблация ультразвуком высокой плотности - 30 пациентов). Результаты. Получены критерии применимости методов термодеструкции. Составлен алгоритм по лечению гиперпаратиреоза, а также подробно рассмотрены методы термодеструкции паращитовидных желез. Заключение. В обзоре анализируется четыре современных метода термодеструкции гиперпаратиреоз, как альтернативы хирургическому вмешательству. Каждый из методов термодеструкции патологически измененных паращитовидных желез имеет преимущества и недостатки, свой профиль эффективности и безопасности. Как показывает анализ существующей доказательной практики, самой большой популярностью среди клиницистов пользуется метод микроволновой аблации, однако более эффективным методом термодеструкции гиперфункционирующих паращитовидных желез является лазерная аблация.

Primary hyperparathyroidism (PHPT) is a common endocrine disease that occurs with multiple profiles in which no classical manifestation. Diagnosis revolves around routine measurement of serum calcium and parathyroid hormone more than in half cases. The understanding of clinical presentation, epidemiology and management tactics of patients with hyperparathyroidism has significantly changed by virtue of the use of biochemical calcium screening. The successful diagnosis and treatment are possible with the cooperation of a multidisciplinary team of endocrinologist, endocrine surgeon, radiologist, nuclear medicine physician and pathomorphologist. The only radical method of treatment is the surgical removal of abnormal parathyroid glands. In this regard, there is necessary to improve the parathyroid glands imaging algorithms. Early treatment of hyperparathyroidism allows to avoid severe damage to the bones, kidneys, heart, other organs, improving the quality of life and reducing the incidence of disability. For a systematic literature review, more than 100 articles published from 2000 to the present time were used, on following resources: PubMed, Embase, SciSearch, Scopus, Cochrane Databases, Research Gate, Google Scholar. Including recommendations from the American Association of Endocrinologists and Endocrine Surgeons (AACE/AAES), European Society of Nuclear Medicine (EANM), European Society of Endocrinologists (ESE), Russian Association of Endocrinologists (RAE) and several other organizations. The main goal of this review is to summarize and present relevant information and a new look on preoperative imaging techniques, methods of intraoperative navigation, surgery, control quality of treatment in patients with primary hyperparathyroidism.

RATIONALE : Insufficient world–wide clinical experience in radioiodine therapy (RIT) for Graves’ disease (GD) in children and adolescents, and limited knowledge of the predictors of RIT efficacy. AIMS : Analysis and identification of the most significant predictors of the efficacy of RIT in children and adolescents with Graves’ disease. MATERIALS AND METHODS : A total of 55 patients (48 females and 7 males) aged from 8 to 18 years receiving primary RIT for GD were enrolled. RIT planning was based on the dosimetric method. Analyzed parameters included gender, age, ultrasound thyroid volume before and 6 months after treatment, the presence of endocrine ophthalmopathy, duration of antithyroid drug (ATD) therapy, relapse of thyrotoxicosis after ATD dose reduction, blood fT3, fT4 and TSH levels initially and at 1, 3, 6 months after treatment, TSH receptor Ab initially and at 3 and 6 months after treatment, thyroid 99m Tc–pertechnetate uptake at 10–20 minutes (%), maximum thyroid 131 I uptake (%), specific 131 I uptake (MBq/g) and therapeutic 131 I activity (MBq). Fisher exact test, non–parametric Mann–Whitney test, Wilcoxon signed–rank test, logistic regression modelling, ROC–analysis, proportional hazard model (the Cox regression), the Kaplan–Meier method and log–rank test were used for statistical analysis as appropriate. RESULTS : Six months after RIT, hypothyroidism was achieved in 45 (81.8%), euthyroid state – in 2 (3.6%), and in 8 (14.6%) patients thyrotoxicosis persisted. On univariate statistical analysis, the smaller thyroid volume, higher fT4 and lower TSH receptor Ab levels, lower 99m Tc–pertechnetate uptake and higher specific 131 I uptake were associated with hypothyroidism. On multivariate logistic regression analysis, the older patient’s age (p=0.011), smaller thyroid volume (p=0.003) and higher fT4 (p=0.024) were independent predictors of RIT efficacy. Thyroid volume was also the only variable associated with achievement of hypothyroidism in time after RIT (p=0.011). CONCLUSION : The efficacy of dosimetry–based RIT in children and adolescents with GD 6 months after treatment was 81.2%. Older patients’ age, smaller thyroid volume and higher fT4 level were independent predictors of therapy success. Smaller thyroid volume was also a predictor of the favorable time–related outcome. Statistical models obtained in this work may be used to prospectively estimate the chance of efficient RIT for GD in pediatric patients.

Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome usually caused by small-sized tumors. Tumors secrete fibroblast growth factor 23 (FGF23), which has a phosphaturic effect. The clinical signs of TIO are non-specific, and include fatigue, bone pain and muscle weakness, which makes timely diagnosis of the disease difficult and treatment is often delayed. Well-timed diagnosis is essential and combined with complete tumor resection it leads to complete relief of symptoms and good postoperative prognosis. In cases of undetected tumors, medical treatment with phosphate supplements and active vitamin D medications is usually successful, however, treatment is associated with numerous complications and side effects can be burdensome for the patients. Due to the risk of recurrence or metastasis, patients with TIO require long-term management and follow-up. In this article, we present a clinical case of successful diagnosis and treatment of TIO in a young patient with type 1 diabetes mellitus.

Medullary thyroid cancer (MTC) is about 5% of all thyroid carcinomas and is hereditary in 20–30% of cases. Multiple endocrine neoplasia syndrome type 2 (MEN2), in addition to medullary thyroid cancer, may include pheochromocytoma, hyperparathyroidism, and some other manifestations. The multidisciplinary data bank (MDB) developed by us is a specialized resource for storing, accumulating and subsequent analysis of data on patients with MTC and MEN2 syndromes. The MDB allows you to collect data from any sources (primary care, medical institutions at various levels, federal centers) and provides internal automated control of the quality and quantity of input data. It is necessary to keep a record of such data to improve the efficiency of diagnostics, treatment and rehabilitation of patients. MDB is an integrated digital platform containing reference materials for doctors, which allows combining evidence-based clinical experience in patients, and provides the opportunity for expert support of medical decisions. In the long run, it will help us organize digitally stored data for machine learning and lays the groundwork for the development of artificial neural networks.

Background. Traditional endogenous stimulation of thyroid-stimulating hormone (TSH) by means of long-term withdrawal of thyroid hormones for radioiodine diagnostics and radioiodine therapy causes severe hypothyroidism, which worsens patients’ general well-being and may lead to side effects and cause tumor growth and dissemination. Exogenous stimulation with recombinant human TSH (rh-TSH, thyrotropin-alfa) causes short-term increases in TSH levels and does not have the above-mentioned side effects. Purpose. To estimate the efficacy and safety of rh-TSH in preparation of patients with well-differentiated thyroid cancer for radioiodine diagnostics and radioiodine therapy. Methods. We conducted an interventional single-center prospective unblinded uncontrolled study of the efficacy and safety of thyrotropin-alfa to prepare patients with well-differentiated thyroid cancer to radioiodine diagnostics and post-surgery radioiodine ablation. The study included 88 patients with well-differentiated thyroid cancer: 54 patients were prepared for post-surgery radioiodine ablation; 34 patients – for radioiodine diagnostics to evaluate combined treatment efficacy and exclusion of tumor recurrence. The level of TSH, thyroglobulin, antibodies to thyroglobulin, whole body scintigraphy, and side effects were measured during exogenous stimulation with thyrotropin-alfa. Results. The level of TSH reached or exceed the target level (30 mIU/ml) 24 hours after the first injection of recombinant thyrotropin-alfa in 86% of patients; after 48 hours in 100%, the level exceeding 100 IU/ml was observed in 66 (75.1%) patients. The maximum levels of thyroglobulin and antibodies to thyroglobulin were reached 72 and 48 hours after the first injection, respectively. The injections of thyrotropin-alfa were well-tolerated by the patients. In the group for radioiodine diagnostics 2 (5.8%) patients complained of fatigue, 1 (2.9%) patient had signs of dyspeptic disorder, while in the group for radioiodine ablation 4 (7.4%) patients complained of fatigue, 1 (1.8%) patient had marked memory problems that disappeared later (they must have been caused by the patient’s advanced age (82 years)). Conclusions. Exogenous recombinant human thyroid-stimulating hormone (thyrotropin-alpha) is highly effective in preparation of patients with well-differentiated thyroid cancer for radioiodine diagnostics and radioiodine ablation. It does not have side effects, which are typical of withdrawal of thyroid hormones. The levels of thyroglobulin and antibodies to thyroglobulin measured 72 hours after the first injection of thyrotropin-alfa have the biggest diagnostic informative value.

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 into 2 groups: the first group – SDH х , SDHAF2 (the assembly factor of SDH, FH, MDH2) – disrupts the Krebs cycle and mitochondrial energy transport chain; the second group – RET, NF1, TMEM127, MAX – leads to mutations in receptor protein kinases (tyrosine kinase), activating intracellular signal pathways (PI3K-AKT-mTOR and MYC), which are responsible for cell growth, growth regulation and cell differentiation. As a result, HIF transcription factors are stabilized (oxidative stress), and DNA methylation is changed, which leads to severe disturbances in gene expression and to malignant transformations of cells. There are three main biochemical phenotypes of PPGLs: noradrenergic, adrenergic and dopaminergic. According to the tumor type, the patient’s age and family history, complementary genetic testing and molecular visualization are recommended. In clinical practice, the biochemical tumor phenotype, its stage, family history and especially the genetic tumor “passport” allow to choose the best molecular visualization method (SPECT-CT/PET-CT) to personalize treatment and prognosis.

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.

Нейроэндокринные опухоли представляют собой гетерогенную группу опухолей, возникающих из энтерохромаффинных клеток диффузной нейроэндокринной системы и встречающихся в 0,5% случаев всех новообразований. За последние годы отмечается значительный рост заболеваемости нейроэндокринными опухолями, что безусловно связано с усовершенствованием методов их диагностики. Однако, несмотря на значительные успехи в изучении биологических и молекулярных механизмов поведения данной группы заболеваний, формирование единого алгоритма диагностики и лечения нейроэндокринных опухолей остается затруднительным и по сей день. Лечение нейроэндокринных опухолей во многом зависит от их функционального статуса и стадии заболевания. Наиболее предпочтительным подходом в лечении локализованных форм нейроэндокринных опухолей остается хирургический. В то же время для больных с распространенными процессами, характеризующимися наличием отдаленных метастазов, на сегодняшний день доступен широкий спектр терапевтических стратегий. Среди них можно выделить контроль над синдромами, ассоциированными с гиперсекрецией тех или иных гормонов, выполнение циторедуктивных вмешательств, системную химиотерапию, применение аналогов соматостатина, а также радиотаргетную терапию. Однако алгоритм выбора того или иного терапевтического подхода в современной клинической практике у разнородной группы больных с нейроэндокринными опухолями требует дальнейшего обсуждения.

Тиреоидит Риделя является редким заболеванием с неустановленной этиологией, которое характеризуется замещением ткани щитовидной железы соединительной, наиболее характерным признаком является каменистая плотность железы, распространяющаяся на окружающие структуры (трахея, пищевод, сосуды и нервы). Плотная спаянность обуславливает клинические симптомы компрессии трахеи, такие как одышка, осиплость голоса, кашель и затруднение дыхания. По функциональной активности может наблюдаться как эутиреоз, так и тиреотоксикоз с последующим развитием гипотиреоза. Заболевание представляет сложности в дооперационной диагностике, которая позволяет лишь предположить данное заболевание, а также дифференциальной диагностике с некоторыми агрессивными формами рака, в связи с чем постановка окончательного диагноза возможна только после проведения гистологического и иммуногистохимического исследования послеоперационного материала. Методами лечения являются консервативный (преимущественно препаратами глюкокортикоидов), а также оперативный. Необходимо щепетильно относиться к выбору метода лечения с учетом плотной спаянности с окружающими структурами и высокого риска развития осложнений оперативного лечения щитовидной железы. Данный клинический случай описывает пациента с подтвержденным морфологическим диагнозом тиреоидита Риделя после проведения тиреоидэктомии. Особенностью является четкая положительная корреляция возникновения субфебрильной лихорадки с дебютом заболевания и ее разрешения после оперативного лечения, срок наблюдения за пациентом составляет 11 мес.

Medullary thyroid cancer with ectopic ACTH production - the disease is extremely rare. The literature describes only a few cases of this disease. Rare ectopic ACTH syndrome caused by medullary thyroid cancer, and the diversity of the clinical picture are responsible for numerous diagnostic errors leading to ineffective treatment. In this regard, we consider it expedient to share our own experience in this area.