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Introduction. Multikinase inhibitors are used to treat nonresectable locally advanced and/or metastatic medullary thyroid cancer (MTC). However they are characterized by high toxicity associated with kinase inhibition. Selective RET inhibitor selpercatinib demonstrates high selectivity and tolerance which makes it a promising agent for MTC treatment.Aim. To evaluate selpercatinib effectiveness and tolerance in patients with metastatic MTC associated with a mutation in the RET gene.Materials and methods. The study included 9 patients with metastatic MTC and mutation in the RET gene who received treatment with selpercatinib 160 mg 2 times a day. The drug effectiveness was evaluated every 2–3 months based on the results of multispiral computed tomography of the whole body and tumor marker (calcitonin and carcinoembryonic antigen) levels.Results. Median duration of therapy was 29 months overall response rate was 78 %; complete response was observed in 56 % of patients. After 12 months of therapy progression-free survival was 100 %; after 24 months it was 89 %. Persistent decrease in calcitonin level (by more than 90 %) was achieved in all patients. The most common adverse events were arterial hypertension and insignificant creatinine increase.Conclusion. The results of therapy show significant improvement in the rate of objective response and progression[1]free survival which makes selpercatinib a preferential treatment choice in this category of patients.

This article discusses the etiology of sporadic and hereditary forms of medullary thyroid cancer (MTC), diagnostic criteria that allow the most accurate and accurate diagnosis and differentiate it from similar symptoms, morphological changes in MTC, which is one of the least studied due to the peculiarities of its histogenetic genesis and low frequency of treatment of the population with this a problem. MTC is often diagnosed at the time of planned hospitalization of patients for problems in a related area, but not directly related to thyroid cancer. In this article, clinical manifestations presented in a specific clinical case are investigated, markers of immunohistochemical studies are determined. This case is interesting and unusual for the duration of the patient's treatment, as well as successful surgical interventions that were aimed at removing multiple metastases. To date, the number of referrals from patients with minimal structural and functional changes in the thyroid gland is small, which indicates that the disease is often diagnosed at a later date. In the minds of a certain percentage of the population, the idea of a possible self-healing of the body is firmly fixed, which is often decisive with the progression of the disease (especially with a sharp jump in progress, for example, after a general somatic decrease in the immune response after infectious diseases, hypothermia, radiation exposure). This article is indicative in this matter – it clearly demonstrates the need for timely vigilance and care for the body, timely medical examination of the population and attentive attitude in particular to the thyroid gland.

A clinical case of examination and treatment of a 29-year-old female patient with type 1 multiple endocrine neoplasia with calcitonin-secreting pancreatic tumors is presented. The difficulties of modern complex diagnostics are shown, as well as the effectiveness of multi-stage surgical tactics involving specialists of various profiles. The conducted laboratory and instrumental examination, which included the entire arsenal of high-tech methods, made it possible to timely diagnose a combined lesion of several endocrine organs. Based on the assessment of the functional activity of the identified tumors, the order of the stages of surgical treatment was substantiated. The implementation of the proposed surgical tactics contributed to the normalization of the patient’s hormonal status and improved quality of life.

Introduction: Medullary thyroid carcinoma is a primary thyroid neoplasm originating from thyroid C cells. It can be familial or sporadic. The familial form is associated with multiple endocrine neoplasia (MEN) syndrome types 2A and 2B and is caused by a mutation in the RET gene, which encodes a tyrosine kinase receptor. Treatment of medullary thyroid cancer is mainly based on surgical resection of the thyroid gland, usually a total thyroidectomy. It can be followed-up by a chemotherapy, which has limited efficacy. Hence, there is a growing interest in new molecular therapies, such as tyrosine kinase inhibitors, which include vandetanib, cabozantinib, selpercatinib, pralsetinib, sorafenib and lenvatinib. Objective: The review and presentation of the current state of knowledge on the systemic treatment of medullary thyroid cancer with kinase inhibitors. Material and methods: Literature review based on available sources from PubMed database and Google Scholar. Conclusions: Though systemic treatment options for medullary thyroid cancer continue to improve, patients with advanced neoplasms still have limited therapeutic options. Hence, further development of targeted treatment with kinase inhibitors, particularly those selective for the RET receptor is crucial. Molecular studies of mutations and signaling pathways involved in the oncopathogenesis of medullary thyroid cancer could contribute to the discovery of new therapeutic mechanisms, and drugs that target them, could potentially further improve disease progression-free survival (PFS) and overall survival (OS).

Background. Coronavirus disease 2019 (COVID-19) is a pandemic of the new millennium. COVID-19 can cause both pulmonary and systemic infammation, and can rapidly progress to multiple organ failure. Data on the relationship between COVID-19 and the thyroid gland have been available since March 2020. The thyroid gland and viral infection as well as associated infammatory-immune reactions participate in a complex interaction. The most common autoimmune disease is chronic autoimmune thyroiditis (chronic lymphocytic thyroiditis, Hashimoto thyroiditis). The majority of medullary thyroid cancers present as a thyroid nodule. Cervical lymph nodes and distant metastases are often detected at the time of diagnosis. The development of autoantibodies may be part of a more complex protective antitumor mechanism, the purpose of which is to eliminate the precursors of future tumor cells.The purpose of the study was to describe a clinical case of diagnosing medullary thyroid cancer in the patient who had COVID-19.Case description. We present the case of a 43-year-old woman who had coronavirus pneumonia (COVID-19), which was accompanied by an increase in serum procalcitonin (PC) level, which required additional examination. Since 2020, she had been observed for autoimmune thyroiditis, which was manifested by a thyroid nodular of a small size. After discharge from the COVID hospital, an ultrasound examination of the thyroid gland revealed an increase in the left lobe thyroid nodule up to 15 mm. The level of calcitonin was 681 pg/ml. Fine needle aspiration (FNA) biopsy of the thyroid gland showed suspicion for medullary thyroid cancer. Thyroidectomy with central lymph node dissection (level VI) was performed. A planned histological examination of the surgical specimen confrmed medullary thyroid cancer.Conclusion. In the present clinical case, medullary thyroid cancer was detected in the patient who had COVID-19 with elevated PC level, which was the basis for a diagnostic search.

The study objective is to perform retrospective analysis of the efficacy and safety of vandetanib for metastatic and non-resectable medullary thyroid cancer in routine clinical practice. Materials and methods. We analyzed treatment outcomes in 46 patients treated with vandetanib. We also evaluated progression-free survival, overall survival, time to progression, and frequency of adverse events. Results. At a median follow-up time of 27.4 months (range: 2.5–106.5 months) and median duration of vandetanib therapy of 21 months, disease progression was registered in 32.6 % of cases, whereas stable disease was observed in 28.3 % of cases and 8.7 % of study participants demonstrated partial response. One patient had complete response to treatment. Almost one-third of patients (28.2 %) died, including 2 individuals whose death was not associated with cancer. The one-year and three-year progression-free survival rates were 67.3 % and 33.3 %, respectively; the two-year and five-year overall survival rates were 82.4 % and 29.4 %, respectively. The efficacy of therapy was confirmed by a 79.4 % decrease in the serum level of calcitonin after treatment initiation. Side effects were observed in 33.9 % of patients (primarily skin and gastrointestinal toxic reactions) and were easily managed in most of the cases. Eight individuals (17.4 %) required cessation of vandetanib due to adverse events. Conclusion. Our findings suggest high efficacy and acceptable safety profile of vandetanib in the treatment of progressive locally advanced non-resectable and disseminated medullary thyroid cancer

Medullary thyroid cancer (MTC) is about 5% of all thyroid carcinomas and is hereditary in 2030% 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.

Medullary thyroid cancer (MTC) is a rare disorder that accounts for approximately 1.7 % of all thyroid malignancies. MTC is usually detected at early stages; however, approximately 10–15 % of patients are diagnosed with locally advanced MTC and distant metastases. Treatment of such patients is challenging due to biological characteristics of the disease and very few effective treatment approaches available. The investigation of mechanisms of carcinogenesis, as well as advances in pharmacology, allowed the development of a new group of targeted drugs, namely tyrosine kinases, which efficacy against progressive unresectable locally advanced or metastatic MTC has been demonstrated in multiple clinical trials. Vandetanib has been registered for MTC treatment in the Russian Federation. MTC is very rare, thus, each case of vandetanib use for its treatment is particularly interesting. Moreover, since the approval of this drug in 2011 by the U. S. Food and Drug Administration (FDA), new data on the clinical use of vandetanib have been accumulated. Importantly, clinical trials are usually well designed and conducted in near-ideal conditions, whereas the real conditions can be different and patients may have individual characteristics. Therefore, the aim of this study was to update the information on the efficacy and safety of vandetanib by retrospective analysis of available publications and to report a case of MTC treated with vandetanib.

There are International and Russian guidelines for the diagnosis and treatment of medullary thyroid carcinoma. There is a recommendation to determine the basal level of calcitonin for all patients with nodular thyroid disease at the all of these documents. However, this test is not performed for this category of patients routinely even in large clinics for a number of reasons, the main one, which seems to be an economic issue. Six months ago a patient addressed to our clinic who underwent surgical treatment for a papillary carcinoma (follicular variant) of the thyroid gland in the volume of thyroidectomy with pre-tracheal lymphodissection and subsequent course of radioiodine therapy at the place of residence. A relapse of the disease was suspected on the control examination at the place of residence and the patient was sent for consultation to the polyclinic of the N.N. Blokhin National Medical Research Center of Oncology. In our clinic, diagnostic studies were carried out, including a revision of the finished cytological and histological preparations and a conclusion was obtained medullary carcinoma of the thyroid. Determination of basal level of calcitonin in serum showed a value of 1292 pg/ml. The level of basal calcitonin significantly decreased after repeated surgical treatment. This case shows that in order to avoid such mistake, which is described in our clinical case, itseems necessary to follow the national recommendations, international standards to determine the level of basal calcitonin for all patients with thyroid nodal pathology who consulted and treated in medical institutions.

Medullary thyroid cancer is a rare tumor. Due to variability of its biological behavior, treatment of this tumor often is a complicated problem. Clinicians have difficulties with determination of indications for prescription of tyrosine kinase inhibitors. Prescription should be based on scru-tinous monitoring of the patients and consideration of all factors: level of tumor markers, patient’s state, and data form visual examinations.

The study objective is to evaluate modern-era radiotherapy (external beam radiotherapy, EBRT) and target therapy (TT) outcomes for advanced medullary thyroid cancer (MTC).Materials and methods. Seventy eight consecutive patients with stage IV MTC were evaluated. All of them with relapsing locally advanced or metastatic MTC, 16 had clinically relevant mediastinal involvement, and 59 had distant metastasis; 26 patients received conformal EBRT or intensity-modulated radiotherapy in monomode, 16 patients – conformal EBRT with simultaneous TT with vandenanib, 36 patients – TT in monomode. Median EBRT dose was 60 Gy.Results. Kaplan–Meier estimates of the median overall survival rate was 14 months for radiotherapy in monomode, 48 months – for conformal EBRT + simultaneous TT with vandenanib, 50 months – for TT in monomode. EBRT and TT allows for significantly shorter periods (median 3.8 weeks), to relieve the symptoms of compression-mediated organs and structures of the neck and mediastinum than in the TT (median 10.2 weeks) (p

Patients who received treatment for the first malignant neoplasm remain in the group at increased risk of developing a second primary tumor throughout their later life. A rare case of medullary thyroid cancer and renal cell carcinoma of the kidney is described in one patient. Medullary thyroid cancer is an orphan disease. The patient and his first-line relatives underwent molecular-genetic testing, the presence of a hereditary form of medullary cancer was excluded. During the observation, malignant kidney formation was detected, and surgical treatment was performed. The patient because of treatment and surveillance does not have data for the progression of thyroid cancer and kidney cancer, compensation for concomitant diseases has been achieved. When observing patients with medullary thyroid cancer, it is necessary to use the existing modern diagnostic arsenal, including positron emission tomography, since patients remain in the group at increased risk of disease progression and development of the second tumor throughout the entire subsequent life. In addition, this case clearly illustrates the need for a routine study of calcitonin as a diagnostic marker for the progression of medullary thyroid cancer.

Medullary thyroid cancer (MTC) is characterized by the most aggressive course among differentiated thyroid carcinomas and is accompanied by early and extensive metastasis. Up to 60 % of patients with MTC already have disseminated forms of the disease (stage III– IV) by the moment of diagnosis. Overall five-year and ten-year survival of patients with MTC is 85–89 and 73–75 %, respectively, whereas five-year survival rate in patients with stage III–IV MTC is significantly lower and does not exceed 5–10 %. Therefore, treatment of disseminated forms of MTC is an extremely important problem in clinical oncology. The article gives an overview of Russian and foreign publications on the effectiveness of radiation and drug therapy for generalized MTC. Particular attention is paid to modern promising treatment strategies used for this category of patients, such as radionuclide therapy of bone metastases and targeted therapy with tyrosine kinase inhibitors.

This article provides our own clinical observation of the patient with multiple endocrine neoplasia type 2B (MEN2B) associated with a germinal mutation in the RET proto-oncogene. Although there are highly informative laboratory methods available, in clinical practice MEN2B syndrome is often diagnosed lately, which results in decrease in patients’ life expectancy and quality.

A corrigendum on Medullary thyroid cancer. Returning to the need to determine the preoperative basal calcitonin level in patients with thyroid nodular pathology by Elena E. Stanyakina, Ilia S. Romanov, Tatiana T. Kondratieva, Alexander S. Krylov, Alexey D. Ryzhkov, Sergey V. Shiryaev (2018). Endocrine Surgery. 12(4). doi: 10.14341/serg10044 There is an error on the page 189: "Blood test for hormones: thyroid stimulating hormone (TSH) 52.6mIU/l (reference values 0.254.0), adrenocorticotropic hormone (ACTH) 20IU/ml (030), thyreoglobulin (TG) 0.2ng/ml (030)". Instead of "antibodies to thyroglobulin (ATTG)" was published "adrenocorticotropic hormone (ACTH)". The authors apologize for this error and state that this does not change the scientific conclusions of the article in any way. The original article has been updated.

MEN 2B syndrome is a subtype of the multiple endocrine neoplasia type 2. It is characterized by the development of aggressive forms of medullary thyroid cancer at an early age, pheochromocytoma and hyperparathyroid syndrome. This article provides an own clinical observation of the patient with MEN 2B syndrome associated with a mutation in the proto-oncogene RET.

In the study were enrolled 137 patients with medullary thyroid cancer (MTC). Low 35%-sensitivity of FNAC and high accuracy of basal calcitonin in MTC diagnostics were found. Mutation analysis of the RET pro- tooncogene in familial and sporadic MTC, RAS -gene in sporadic MTC were done. The correlation between type of the mutation and disease aggressiveness was found.

During last 4 years leading endocrine societies of the world published clinical recommendations on diag nostics and treatment of medullary thyroid cancer. The article covers most aspects of following patients with this pathology.

Distant metastases are the main cause of death in patients with medullary thyroid cancer (MTC). These 21 recommendations focus on MTC patients with distant metastases and a detailed followup protocol of patients with biochemical or imaging evidence of disease, selection criteria for treatment, and treatment modalities, including local and systemic treatments based on the results of recent trials. Asymptomatic patients with low tumor burden and stable disease may benefit from local treatment modalities and can be followed up at regular intervals of time. Imaging is usually performed every 6–12 months, or at longer inter vals of time depending on the doubling times of serum calcitonin and carcinoembryonic antigen levels. Patients with symptoms, large tumor burden and progression on imaging should receive systemic treatment. Indeed, major progress has recently been achieved with novel targeted therapies using kinase inhibitors directed against RET and VEGFR, but further research is needed to improve the outcome of these patients.

Medullary thyroid cancer (MTC) has high level of clinical aggressiveness as well as high recurrence rate par ticularly metastases in regional lymph nodes. Surgery is single curative treatment method thus only initial appropriate operation volume could improve diseasefree and overall survival. In our study the experience of surgical treatment of 125 MTC patients is analyzing. In 25% of cases there were familial MTC (germline RET/MTC mutation verified) disease and in 75% – sporadic tumors. Comparison of tenyears diseasefree sur vival in two groups with various initial surgery extensions revealed that thyroidectomy associated with central neck dissection as a minimal operation volume has significantly improved longterm diseasefree survival as against to lesser initial surgery extension.

Medullary thyroid cancer (MTC) is a fairly rare disease, accounting for 5-7% of all cases of thyroid cancer. The sporadic form of thyroid cancer is observed in 70-80% of cases, family (inherited - autosomal dominant type of inheritance) - in 20-30%. Familial forms of MTCG are caused by point mutations in the RET proto-oncogen (Rearranged during Transfection). To date, about 25 germinal (inherited) mutations are described in the world literature in 19 codons of the RET gene, which are found in 97% of patients with MEN 2A, in 95% with MEN 2B and in 86% of patients with SMR.