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<article article-type="research-article" dtd-version="1.3" 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" xml:lang="ru"><front><journal-meta><journal-id journal-id-type="publisher-id">nodgo</journal-id><journal-title-group><journal-title xml:lang="ru">Российский журнал детской гематологии и онкологии (РЖДГиО)</journal-title><trans-title-group xml:lang="en"><trans-title>Russian Journal of Pediatric Hematology and Oncology</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2311-1267</issn><issn pub-type="epub">2413-5496</issn><publisher><publisher-name>LTD “Graphica”</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.21682/2311-1267-2025-12-3-103-112</article-id><article-id custom-type="elpub" pub-id-type="custom">nodgo-1194</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>CLINICAL CASES</subject></subj-group></article-categories><title-group><article-title>RET-перестроенная веретеноклеточная саркома мягких тканей у детей: описание клинического случая и обзор литературы</article-title><trans-title-group xml:lang="en"><trans-title>RET-rearranged spindle cell soft tissue sarcoma in children: description of a clinical case and literature review</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0000-2670-547X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Зацаринная</surname><given-names>О. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Zatsarinnaya</surname><given-names>O. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Ольга Сергеевна Зацаринная - Врач-детский онколог отделения клинической онкологии НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Pediatric Oncologist of the Department of Clinical Oncology.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">clinoncology@list.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1308-8622</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Друй</surname><given-names>А. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Druy</surname><given-names>A. E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заведующий лабораторией молекулярной онкологии НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Head of the Laboratory of Molecular Oncology.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">alexander.druy@fccho-moscow.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7732-8184</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Коновалов</surname><given-names>Д. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Konovalov</surname><given-names>D. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заведующий отделением патологической анатомии НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Head of the Pathology Department.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">dmk_nadf@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-5626-218X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Андреева</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Andreeva</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., врач-детский онколог отделения клинической онкологии НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Pediatric Oncologist Department of Clinical Oncology.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">andreeva793@bk.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-8580-3499</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Панферова</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Panferova</surname><given-names>A. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.б.н., старший научный сотрудник лаборатории цитогенетики и молекулярной генетики НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Biol.), Senior Researcher Laboratory of Cytogenetics and Molecular Genetics.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">agnesa.panferova@dgoi.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-5354-7067</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шарлай</surname><given-names>А. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Sharlai</surname><given-names>A. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Врач-лабораторный генетик отделения патологической анатомии НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Laboratory Geneticist Pathological Anatomical Department.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">anastasia.sharlai@dgoi.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1661-4205</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Синиченкова</surname><given-names>К. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Sinichenkova</surname><given-names>K. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Врач-детский онколог отделения онкологии и гематологии старшего возраста НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Pediatric Oncologist, Department of Oncology and Hematology for the Elderly.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">kseniya.sinichenkova@dgoi.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3767-4477</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шаманская</surname><given-names>Т. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Shamanskaya</surname><given-names>T. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., врач-детский онколог, заведующая отделом изучения эмбриональных опухолей Института онкологии, радиологии и ядерной медицины НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Head of the Department of Embryonal Tumors, Institute of Oncology, Radiology and Nuclear Medicine.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">Tatyana.Shamanskaya@dgoi.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9375-7517</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Рощин</surname><given-names>В. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Roshchin</surname><given-names>V. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Врач-патологоанатом патологоанатомического отделения НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Pathologist of the Pathology Department.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">vitaly.roschin@dgoi.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-8129-0545</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Щербаков</surname><given-names>А. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Shcherbakov</surname><given-names>A. P.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Врач-рентгенолог рентгенологического отделения НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Radiologist Radiology Department.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">Alexey.Shcherbakov@dgoi.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6313-6712</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Большаков</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Bolshakov</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заведующий отделением онкологии и детской хирургии, руководитель ортопедической хирургической группы НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Head of the Department of Oncology and Pediatric Surgery, Head of the Orthopedic Surgical Group.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">bolshakovn@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3704-8783</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Качанов</surname><given-names>Д. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Kachanov</surname><given-names>D. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., заместитель директора Института онкологии, радиологии и ядерной медицины и заведующий отделением клинической онкологии НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Deputy Director of the Institute of Oncology, Radiology and Nuclear Medicine &amp; Head of the Department of Clinical Oncology.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">denis.kachanov@dgoi.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2025</year></pub-date><pub-date pub-type="epub"><day>02</day><month>11</month><year>2025</year></pub-date><volume>12</volume><issue>3</issue><fpage>103</fpage><lpage>112</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Зацаринная О.С., Друй А.Е., Коновалов Д.М., Андреева Н.А., Панферова А.В., Шарлай А.С., Синиченкова К.Ю., Шаманская Т.В., Рощин В.Ю., Щербаков А.П., Большаков Н.А., Качанов Д.Ю., 2025</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="ru">Зацаринная О.С., Друй А.Е., Коновалов Д.М., Андреева Н.А., Панферова А.В., Шарлай А.С., Синиченкова К.Ю., Шаманская Т.В., Рощин В.Ю., Щербаков А.П., Большаков Н.А., Качанов Д.Ю.</copyright-holder><copyright-holder xml:lang="en">Zatsarinnaya O.S., Druy A.E., Konovalov D.M., Andreeva N.A., Panferova A.V., Sharlai A.S., Sinichenkova K.Y., Shamanskaya T.V., Roshchin V.Y., Shcherbakov A.P., Bolshakov N.A., Kachanov D.Y.</copyright-holder><license xml:lang="ru" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>Данная работа распространяется под лицензией Creative Commons Attribution 4.0.</license-p></license><license xml:lang="en" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>This work is licensed under a Creative Commons Attribution 4.0 License.</license-p></license></permissions><self-uri xlink:href="https://journal.nodgo.org/jour/article/view/1194">https://journal.nodgo.org/jour/article/view/1194</self-uri><abstract><p>Саркомы мягких тканей (СМТ) представляют собой гетерогенную группу злокачественных опухолей мезенхимального происхождения. Широкое внедрение в клиническую практику методов секвенирования нового поколения, включая секвенирование РНК, привело к описанию целого ряда СМТ с перестройками генов, кодирующих различные киназы.</p><p>В настоящей статье описывается клинический случай пациента 3 лет с веретеноклеточной саркомой низкой степени злокачественности с выявленным РНК-транскриптом MYH10::RET. В обзоре литературы описывается роль гена RET в патогенезе новообразований с акцентом на опухоли мягких тканей, представлена клиническая, молекулярно-генетическая характеристики педиатрических случаев СМТ с перестройками гена RET и результаты их терапии. Обсуждается роль различных методов молекулярной онкологии в идентификации перестроек генов киназ, включая RET.</p></abstract><trans-abstract xml:lang="en"><p>Soft tissue sarcomas (SMTs) represent a heterogeneous group of malignant tumors of mesenchymal origin. The widespread introduction of next-generation sequencing techniques, including RNA sequencing, into clinical practice has led to the description of a variety of SMTs with rearrangements of genes encoding various kinases.</p><p>The present article describes the clinical case of a 3-year-old patient with a low-grade spindle cell sarcoma with a detectable MYH10::RET RNA transcript. The literature review describes the role of the RET gene in the pathogenesis of malignant neoplasms, with emphasis on soft tissue tumors, and presents the clinical, molecular and genetic characteristics of pediatric cases of SMT with RET gene rearrangements and the results of their therapy. The role of various molecular oncology techniques in the identification of kinase gene rearrangements, including RET, is discussed.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>саркомы мягких тканей</kwd><kwd>дети</kwd><kwd>ген RET</kwd><kwd>транслокации</kwd></kwd-group><kwd-group xml:lang="en"><kwd>soft tissue sarcomas</kwd><kwd>children</kwd><kwd>RET gene</kwd><kwd>translocations</kwd></kwd-group><funding-group><funding-statement xml:lang="ru">Исследование проведено без спонсорской поддержки</funding-statement><funding-statement xml:lang="en">The study was performed without external funding</funding-statement></funding-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">WHO Classifi cation of Tumours of Soft Tissue and Bone, 5th ed. Lyon, France: IARC Press, 2020.</mixed-citation><mixed-citation xml:lang="en">WHO Classifi cation of Tumours of Soft Tissue and Bone, 5th ed. 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