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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="other" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Bone and soft tissue sarcomas, tumors of the skin</journal-id><journal-title-group><journal-title xml:lang="en">Bone and soft tissue sarcomas, tumors of the skin</journal-title><trans-title-group xml:lang="ru"><trans-title>Саркомы костей, мягких тканей и опухоли кожи</trans-title></trans-title-group></journal-title-group><issn publication-format="print">2219-4614</issn><issn publication-format="electronic">2782-3687</issn><publisher><publisher-name xml:lang="en">Publishing House ABV Press</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">110</article-id><article-id pub-id-type="doi">10.17650/2070-9781-2021-13-1-24-31</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>BONE SARCOMAS</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>САРКОМЫ КОСТЕЙ</subject></subj-group><subj-group subj-group-type="article-type"><subject></subject></subj-group></article-categories><title-group><article-title xml:lang="en">Molecular, biological and diagnostic features of Ewing sarcoma and undifferentiated small round cell sarcomas of bone and soft tissue</article-title><trans-title-group xml:lang="ru"><trans-title>Молекулярно-биологические и диагностические особенности саркомы Юинга и группы недифференцированных мелкокруглоклеточных опухолей костей и мягких тканей</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1661-4205</contrib-id><name-alternatives><name xml:lang="en"><surname>Sinichenkova</surname><given-names>K. Y.</given-names></name><name xml:lang="ru"><surname>Синиченкова</surname><given-names>К. Ю.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>1 Samory Mashela St., Moscow 117198</p></bio><bio xml:lang="ru"><p>117198 Москва, ул. Саморы Машела, 1</p></bio><email>ksinichenkova@gmail.com</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9375-7517</contrib-id><name-alternatives><name xml:lang="en"><surname>Roschin</surname><given-names>V. Y.</given-names></name><name xml:lang="ru"><surname>Рощин</surname><given-names>В. Ю.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>1 Samory Mashela St., Moscow 117198</p></bio><bio xml:lang="ru"><p>117198 Москва, ул. Саморы Машела, 1</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1308-8622</contrib-id><name-alternatives><name xml:lang="en"><surname>Druy</surname><given-names>A. E.</given-names></name><name xml:lang="ru"><surname>Друй</surname><given-names>А. Е.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>1 Samory Mashela St., Moscow 117198</p></bio><bio xml:lang="ru"><p>117198 Москва, ул. Саморы Машела, 1</p></bio><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology, Ministry of Healh of Russia</institution></aff><aff><institution xml:lang="ru">ФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2021-06-03" publication-format="electronic"><day>03</day><month>06</month><year>2021</year></pub-date><volume>13</volume><issue>1</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>24</fpage><lpage>31</lpage><history><date date-type="received" iso-8601-date="2021-06-02"><day>02</day><month>06</month><year>2021</year></date><date date-type="accepted" iso-8601-date="2021-06-02"><day>02</day><month>06</month><year>2021</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2021, Sinichenkova K.Y., Roschin V.Y., Druy A.E.</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2021, Синиченкова К.Ю., Рощин В.Ю., Друй А.Е.</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="en">Sinichenkova K.Y., Roschin V.Y., Druy A.E.</copyright-holder><copyright-holder xml:lang="ru">Синиченкова К.Ю., Рощин В.Ю., Друй А.Е.</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://sarbon.abvpress.ru/jour/article/view/110">https://sarbon.abvpress.ru/jour/article/view/110</self-uri><abstract xml:lang="en"><p>Ewing’s sarcoma is a highly malignant small round cell tumor with a unique rearrangement of the EWSR1 (FUS) gene with partners genes of ETS family. Tumors with Ewing's sarcoma morphological features lacking without specific EWSR1 rear-rangement called undifferentiated small round cell sarcomas of bone and soft tissue. This group includes: sarcomas with СIC gene rearrangement, sarcomas with BCOR gene rearrangement and sarcomas with EWSR1 (FUS) gene rearrangement with non-ETS gene-partner. Clinical, morphological and molecular genetic characteristics of these groups of tumors will be described below</p></abstract><trans-abstract xml:lang="ru"><p>Саркома Юинга – высокозлокачественная мелкокруглоклеточная опухоль, обладающая уникальной перестройкой гена EWSR1 (FUS) с генами-партнерами семейства ETS. Опухоли, обладающие морфологическими характеристиками саркомы Юинга, но не имеющие патогномоничной перестройки, входят в группу недифференцированных мелкокруглоклеточных опухолей костей и мягких тканей, куда также относятся саркомы с перестройкой генов CIC, BCOR, EWSR1 (FUS) с другими генами, не являющимися членами семейства ETS. Ниже будут изложены клинико-морфологические и молекулярно-генетические особенности данных групп злокачественных новообразований.</p></trans-abstract><kwd-group xml:lang="en"><kwd>Ewing sarcoma</kwd><kwd>undifferentiated small round cell sarcomas</kwd><kwd>EWSR1</kwd><kwd>FUS</kwd><kwd>СIC-DUX4</kwd><kwd>BCOR-CCNB3</kwd><kwd>undifferentiated sarcomas</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>саркома Юинга</kwd><kwd>недифференцированные мелкокруглоклеточные саркомы</kwd><kwd>EWSR1</kwd><kwd>FUS</kwd><kwd>СIC-DUX4</kwd><kwd>BCOR-CCNB3</kwd><kwd>недифференцированные саркомы</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><citation-alternatives><mixed-citation xml:lang="en">1. Parham D.M. Modern diagnosis of small cell malignancies of children. Surg Patholog Clinics 2010;3(3):515–51. DOI: 10.1016/j.path.2010.06.002.</mixed-citation><mixed-citation xml:lang="ru">Parham D.M. 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