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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">Annals of the Russian academy of medical sciences</journal-id><journal-title-group><journal-title xml:lang="en">Annals of the Russian academy of medical sciences</journal-title><trans-title-group xml:lang="ru"><trans-title>Вестник Российской академии медицинских наук</trans-title></trans-title-group></journal-title-group><issn publication-format="print">0869-6047</issn><issn publication-format="electronic">2414-3545</issn><publisher><publisher-name xml:lang="en">"Paediatrician" Publishers LLC</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">2132</article-id><article-id pub-id-type="doi">10.15690/vramn2132</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>ENDOCRINOLOGY: CURRENT ISSUES</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>АКТУАЛЬНЫЕ ВОПРОСЫ ЭНДОКРИНОЛОГИИ</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">Treatment of Parathyroid Cancer: Current Status and Near-Term Prospects</article-title><trans-title-group xml:lang="ru"><trans-title>Лечение рака околощитовидных желез: текущее состояние и ближайшие перспективы</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7879-8495</contrib-id><contrib-id contrib-id-type="spin">1628-2139</contrib-id><name-alternatives><name xml:lang="en"><surname>Kim</surname><given-names>Ekaterina I.</given-names></name><name xml:lang="ru"><surname>Ким</surname><given-names>Екатерина Игоревна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, PhD Student</p></bio><bio xml:lang="ru"><p>врач-эндокринолог, аспирант</p></bio><email>kat-alex2007@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7963-5022</contrib-id><contrib-id contrib-id-type="spin">6279-8247</contrib-id><name-alternatives><name xml:lang="en"><surname>Krupinova</surname><given-names>Julia A.</given-names></name><name xml:lang="ru"><surname>Крупинова</surname><given-names>Юлия Александровна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, PhD</p></bio><bio xml:lang="ru"><p>врач-эндокринолог, к.м.н.</p></bio><email>j.krupinova@gmail.com</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-8216-517X</contrib-id><contrib-id contrib-id-type="spin">5531-6435</contrib-id><name-alternatives><name xml:lang="en"><surname>Belousov</surname><given-names>Pavel V.</given-names></name><name xml:lang="ru"><surname>Белоусов</surname><given-names>Павел Владимирович</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Senior Research Associate</p></bio><bio xml:lang="ru"><p>старший научный сотрудник</p></bio><email>belousp@gmail.com</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4748-6362</contrib-id><name-alternatives><name xml:lang="en"><surname>Trutneva</surname><given-names>Kseniya А.</given-names></name><name xml:lang="ru"><surname>Трутнева</surname><given-names>Ксения Александровна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Researcher, PhD in Biology</p></bio><bio xml:lang="ru"><p>научный сотрудник, к.б.н.</p></bio><email>trutneva-k@mail.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9717-9742</contrib-id><contrib-id contrib-id-type="spin">5624-3875</contrib-id><name-alternatives><name xml:lang="en"><surname>Mokrysheva</surname><given-names>Natalya G.</given-names></name><name xml:lang="ru"><surname>Мокрышева</surname><given-names>Наталья Георгиевна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, PhD, Professor</p></bio><bio xml:lang="ru"><p>д.м.н., профессор</p></bio><email>mokrisheva.natalia@endocrincentr.ru</email><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Endocrinology Research Centre</institution></aff><aff><institution xml:lang="ru">Национальный медицинский исследовательский центр эндокринологии</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2022-12-28" publication-format="electronic"><day>28</day><month>12</month><year>2022</year></pub-date><volume>77</volume><issue>5</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>362</fpage><lpage>370</lpage><history><date date-type="received" iso-8601-date="2022-06-27"><day>27</day><month>06</month><year>2022</year></date><date date-type="accepted" iso-8601-date="2022-11-03"><day>03</day><month>11</month><year>2022</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2022, "Paediatrician" Publishers LLC</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2022, Издательство "Педиатръ"</copyright-statement><copyright-year>2022</copyright-year><copyright-holder xml:lang="en">"Paediatrician" Publishers LLC</copyright-holder><copyright-holder xml:lang="ru">Издательство "Педиатръ"</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/" start_date="2023-12-28"/></permissions><self-uri xlink:href="https://vestnikramn.spr-journal.ru/jour/article/view/2132">https://vestnikramn.spr-journal.ru/jour/article/view/2132</self-uri><abstract xml:lang="en"><p><italic>Parathyroid cancer (PС) is a rare malignant neoplasm of the endocrine system, characterized by a severe course due to the development of life-threatening hypercalcemia. Despite a relatively high survival rate, recurrence occurs about half the time. The aggressive course of PС is associated with both local spread of the process and distant metastasis. With the exception of isolated cases, cytotoxic chemo- and radiotherapy are inefficacious, and treatment protocols have not been developed. Therefore the only effective way is the surgical removal of secondary lesions, which is not always technically feasible and is associated with a high risk of postoperative complications. The literature describes individual cases of successful use of targeted therapy, as well as immunotherapy in the form of therapeutic vaccination to induce an immune response against parathyroid hormone (PTH) and block immune checkpoint. A promising direction of study is the tumor immunoenvironment, which has both diagnostic and prognostic value in relation to the likelihood of the spread of the tumor process. In this review, we describe the main directions in the treatment of PC, including disseminated forms, the possibilities of immunotherapy will be considered in details.</italic></p></abstract><trans-abstract xml:lang="ru"><p><italic>Рак околощитовидных желез (ОЩЖ) — редкое злокачественное новообразование эндокринной системы, характеризующееся тяжелым течением вследствие развития жизнеугрожающей гиперкальциемии. Несмотря на относительно высокую выживаемость, рецидив возникает примерно в половине случаев. Агрессивное течение рака ОЩЖ ассоциировано как с локальным распространением процесса, так и с отдаленным метастазированием. За исключением единичных случаев, эффективность цитотоксической химио- и лучевой терапии при лечении рака ОЩЖ низкая, а протоколы лечения не разработаны, поэтому основным способом лечения остается хирургическое удаление вторичных очагов, что не всегда технически выполнимо и сопряжено с высоким риском послеоперационных осложнений. В литературе описаны отдельные случаи успешного применения таргетной терапии, а также иммунотерапии в формате терапевтической вакцинации с целью индукции иммунного ответа против паратиреоидного гормона (ПТГ) и блокировки контрольных точек иммунного ответа. Перспективное направление — изучение иммуноокружения опухоли, которое имеет как диагностическую, так и прогностическую ценность в отношении вероятности распространения опухолевого процесса. Данный обзор посвящен основным направлениям в лечении рака ОЩЖ, в том числе диссеминированных форм, подробно рассмотрены возможности и перспективы иммунотерапии.</italic></p></trans-abstract><kwd-group xml:lang="en"><kwd>parathyroid cancer</kwd><kwd>neoplasm metastasis</kwd><kwd>immunotherapy</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>рак околощитовидных желез</kwd><kwd>метастазы</kwd><kwd>иммунотерапия</kwd></kwd-group><funding-group><award-group><funding-source><institution-wrap><institution xml:lang="ru">Правительство Российской Федерации</institution></institution-wrap><institution-wrap><institution xml:lang="en">Government of the Russian Federation</institution></institution-wrap></funding-source><award-id>121030100032-7</award-id></award-group></funding-group></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Hundahl SA, Fleming ID, Fremgen AM, et al. 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