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<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="review-article" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Russian Journal of Oncology</journal-id><journal-title-group><journal-title xml:lang="en">Russian Journal of Oncology</journal-title><trans-title-group xml:lang="ru"><trans-title>Российский онкологический журнал</trans-title></trans-title-group></journal-title-group><issn publication-format="print">1028-9984</issn><issn publication-format="electronic">2412-9119</issn><publisher><publisher-name xml:lang="en">Eco-Vector</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">40006</article-id><article-id pub-id-type="doi">10.17816/onco40006</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>Articles</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>Статьи</subject></subj-group><subj-group subj-group-type="article-type"><subject>Review Article</subject></subj-group></article-categories><title-group><article-title xml:lang="en">HEREDITARY MEDULLARY THYROID CANCER: TREATMENT, FOLLOW-UP, PROGNOSIS (PART II)</article-title><trans-title-group xml:lang="ru"><trans-title>НАСЛЕДСТВЕННЫЙ МЕДУЛЛЯРНЫЙ РАК ЩИТОВИДНОЙ ЖЕЛЕЗЫ: ЛЕЧЕНИЕ, НАБЛЮДЕНИЕ, ПРОГНОЗ ( часть II)</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Yukina</surname><given-names>M. Yu</given-names></name><name xml:lang="ru"><surname>Юкина</surname><given-names>Марина Юрьевна</given-names></name></name-alternatives><bio xml:lang="ru"><p>канд. мед. наук, ст. научн. сотр. отд-ния терапии</p></bio><email>endo-yukina@yandex.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Troshina</surname><given-names>E. A</given-names></name><name xml:lang="ru"><surname>Трошина</surname><given-names>Е. А</given-names></name></name-alternatives><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Beltsevich</surname><given-names>D. G</given-names></name><name xml:lang="ru"><surname>Бельцевич</surname><given-names>Д. Г</given-names></name></name-alternatives><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Rumyantsev</surname><given-names>P. O</given-names></name><name xml:lang="ru"><surname>Румянцев</surname><given-names>П. О</given-names></name></name-alternatives><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Federal Endocrinological Research Center</institution></aff><aff><institution xml:lang="ru">ФГБУ «Эндокринологический научный центр» Минздрава России</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2013-08-15" publication-format="electronic"><day>15</day><month>08</month><year>2013</year></pub-date><volume>18</volume><issue>4</issue><issue-title xml:lang="en">NO4 (2013)</issue-title><issue-title xml:lang="ru">№4 (2013)</issue-title><fpage>25</fpage><lpage>30</lpage><history><date date-type="received" iso-8601-date="2020-07-22"><day>22</day><month>07</month><year>2020</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2013, Eco-Vector</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2013, ООО "Эко-Вектор"</copyright-statement><copyright-year>2013</copyright-year><copyright-holder xml:lang="en">Eco-Vector</copyright-holder><copyright-holder xml:lang="ru">ООО "Эко-Вектор"</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/></permissions><self-uri xlink:href="https://rjonco.com/1028-9984/article/view/40006">https://rjonco.com/1028-9984/article/view/40006</self-uri><abstract xml:lang="en"><p>In hereditary MTC recommended age surgery in carriers of the mutation depends on its localization in the gene RET, which determines the probable age of tumor onset, aggressiveness and prognosis of its clinical course. The optimal treatment of the carrier RET mutation is generally recognized preventive thyroidectomy in childhood before the possible onset of malignancy. The only radical treatment of MTC in operable patients is surgical. Regardless of the etiology ( sporadic, familial) MTC is prone to early and extensive metastatic dissemination. The minimum volume of transactions in medullary carcinoma is total thyroidectomy, supplemented with central lymph node dissection. When tumor is operable and in the absence of data for distant metastases should seek to limit the surgical radicality. Clinical supervision with recurrent dinamics assessment of basal and stimulated calcitonin and carcinoembryonic antigen level after the surgery is indicated. In inoperable cases and after progression of distant metastases treatment options are extremely limited. MTC is a radiosensitive tumor. Chemotherapy has shown very limited effectiveness under high toxicity and therefore is not assigned. Satisfactory anti-tumor effect in moderate toxicity showed some tyrosine kinase inhibitors on the results of recent randomized clinical trials.</p></abstract><trans-abstract xml:lang="ru"><p>При наследственном мездуллярном раке щитовидной железы (МРЩЖ) рекомендуемый возраст операции у носителя мутации зависит от ее локализации в гене RET, которая предопределяет вероятный возраст манифестации опухоли и прогноз агрессивности ее клинического течения. Оптимальным лечением носителя RET-мутации общепризнана превентивная тиреоидэктомия в детском возрасте до возможного начала малигнизации. Единственным радикальным методом лечения МРЩЖ у операбельных пациентов является хирургический. Вне зависимости от этиологии (спорадический, наследственный) МРЩЖ склонен к ранней и обширной метастатической диссеминации. Минимальным объемом операции при медуллярной карциноме считается тотальная тиреоидэктомия, дополненная центральной лимфодиссекцией. При резектабельно-сти опухолевого процесса и в отсутствии данных, указывающих на отдаленные метастазы, необходимо стремиться к предельной хирургической радикальности. После операции показано диспансерное наблюдение с периоидической оценкой динамики уровней базального и стимулированного кальцитонина, а также раковоэмбрионального антигена. В неоперабельных случаях и при развитии отдаленных метастазов выбор методов лечения крайне ограничен. МРЩЖ не является радиочувствительной опухолью. Химиотерапия дала весьма ограниченную эффективность при высокой токсичности и поэтому не назначается. Удовлетворительный противоопухолевый эффект при умеренной токсичности продемонстрировали отдельные ингибиторы тирозинкиназ по результатам недавно проведенных рандомизированных клинических исследований.</p></trans-abstract><kwd-group xml:lang="en"><kwd>RET</kwd><kwd>medullary thyroid cancer</kwd><kwd>RET</kwd><kwd>surgery</kwd><kwd>calcitonin</kwd><kwd>tyrosine kinase inhibitors</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>медуллярный рак щитовидной железы</kwd><kwd>хирургическое лечение</kwd><kwd>кальцитонин</kwd><kwd>ингибиторы тирозинкиназ</kwd></kwd-group></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Quayle F.J., Moley J.F. Medullary thyroid carcinoma, including MEN 2A and MEN 2B syndromes. J. Surg. Oncol. 2005; 89 (3): 122-9.</mixed-citation></ref><ref id="B2"><label>2.</label><mixed-citation>Brierley J., Tsang R., Simpson W.J., Gospodarowicz M., Sutcliffe S., Panzarella T. 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