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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">10.21682/2311-1267-2025-12-3-85-94</article-id><article-id custom-type="elpub" pub-id-type="custom">nodgo-1192</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>Эмбриональная опухоль центральной нервной системы с амплификацией PLAGL1: описание клинического случая и обзор литературы</article-title><trans-title-group xml:lang="en"><trans-title>Embryonal tumor of the central nervous system with PLAGL1 amplification: a case report 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/0000-0002-7531-6443</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ольхова</surname><given-names>Л. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Olkhova</surname><given-names>L. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Людмила Владимировна Ольхова - к.м.н., врач-детский онколог отделения трансплантации костного мозга РДКБ – филиала РНИМУ им. Н.И. Пирогова.</p><p>119571, Москва, Ленинский просп., 117</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Pediatric Oncologist Department of Bone Marrow Transplantation, Russian Children’s Clinical Hospital – Branch of the N.I. Pirogov Russian National Research Medical University of Ministry of Health of Russia.</p><p>117 Leninskiy Prosp., Moscow, 119571</p></bio><email xlink:type="simple">rylkova87@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/0000-0002-8607-3635</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Желудкова</surname><given-names>О. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Zheludkova</surname><given-names>O. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., профессор, эксперт ВАК по детской нейроонкологии, главный научный сотрудник НПЦ спец. мед. помощи детям ДЗМ, врач-детский онколог ООО «ЛДЦ МИБС».</p><p>119620, Москва, ул. Авиаторов, 38; 194354, Санкт-Петербург, ул. Есенина, 2, корп. 3</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Professor, Expert of the Higher Attestation Commission on Pediatric Neuro-Oncology, Chief Researcher of V.F. Voyno-Yasenetsky Scientific and Practical Center of Specialized Medical Care for Children, Pediatric Oncologist DTC IIBS named after S. Berezin.</p><p>38 Aviatorov St., Moscow, 119620; Bldg. 3, 2 Yesenin St., S.-Petersburg, 194354</p></bio><email xlink:type="simple">clelud@mail.ru</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7206-6365</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Рыжова</surname><given-names>М. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Ryzhova</surname><given-names>M. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., заведующая патологоанатомическим отделением НМИЦ нейрохирургии им. акад. Н.Н. Бурденко.</p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Head of the Pathology Department.</p><p>16 4th Tverskaya-Yamskaya St., Moscow, 125047</p></bio><email xlink:type="simple">mrizhova@nsi.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7136-0693</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кушель</surname><given-names>Ю. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Kushel</surname><given-names>Yu. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., профессор, врач-нейрохирург 2-го нейрохирургического отделения НМИЦ нейрохирургии им. акад. Н.Н. Бурденко.</p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Professor, Neurosurgeon of 2nd Children Neurosurgical Department.</p><p>16 4th Tverskaya-Yamskaya St., Moscow, 125047</p></bio><email xlink:type="simple">kuszel@yandex.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7045-7223</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шишкина</surname><given-names>Л. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Shishkina</surname><given-names>L. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., врач-патологоанатом патологоанатомического отделения НМИЦ нейрохирургии им. акад. Н.Н. Бурденко.</p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Pathologist Pathology Department.</p><p>16 4th Tverskaya-Yamskaya St., Moscow, 125047</p></bio><email xlink:type="simple">lshishkina@nsi.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1898-7769</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Романов</surname><given-names>П. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Romanov</surname><given-names>P. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заведующий отделением лучевой диагностики НПЦ спец. мед. помощи детям ДЗМ.</p><p>119620, Москва, ул. Авиаторов, 38</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Head of the Pathology Department.</p><p>38 Aviatorov St., Moscow, 119620</p></bio><email xlink:type="simple">nptc_xray@mail.ru</email><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1664-7103</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Постникова</surname><given-names>Т. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Postnikova</surname><given-names>T. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Врач-детский онколог онкологического отделения № 2 НПЦ спец. мед. помощи детям ДЗМ.</p><p>119620, Москва, ул. Авиаторов, 38</p></bio><bio xml:lang="en"><p>Pediatric Oncologist 2nd Oncology Department of the Scientific and Practical Center for Specialized Medical Care for Children named after V.F. Voyno-Yasenetsky Moscow Healthcare Department.</p><p>38 Aviatorov St., Moscow, 119620</p></bio><email xlink:type="simple">t.postnikova1984@mail.ru</email><xref ref-type="aff" rid="aff-4"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>РДКБ – филиал ФГАОУ ВО РНИМУ им. Н.И. Пирогова Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Russian Children’s Clinical Hospital – Branch of the N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ГБУЗ «НПЦ спец. мед. помощи детям ДЗМ»; ООО «ЛДЦ МИБС»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>V.F. Voyno-Yasenetsky Scientific and Practical Center of Specialized Medical Care for Children; Diagnostic and Treatment Center of International Institution for Biological Systems named after Sergey Berezin</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>ФГАУ «НМИЦ нейрохирургии им. акад. Н.Н. Бурденко» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.N. Burdenko National Medical Research Center of Neurosurgery, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>ГБУЗ «НПЦ спец. мед. помощи детям ДЗМ»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>V.F. Voyno-Yasenetsky Scientific and Practical Center of Specialized Medical Care for Children</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>85</fpage><lpage>94</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Ольхова Л.В., Желудкова О.Г., Рыжова М.В., Кушель Ю.В., Шишкина Л.В., Романов П.А., Постникова Т.В., 2025</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="ru">Ольхова Л.В., Желудкова О.Г., Рыжова М.В., Кушель Ю.В., Шишкина Л.В., Романов П.А., Постникова Т.В.</copyright-holder><copyright-holder xml:lang="en">Olkhova L.V., Zheludkova O.G., Ryzhova M.V., Kushel Y.V., Shishkina L.V., Romanov P.A., Postnikova T.V.</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/1192">https://journal.nodgo.org/jour/article/view/1192</self-uri><abstract><p>Эмбриональная опухоль центральной нервной системы (ЦНС) с амплификацией PLAGL1/PLAGL2 – это новый тип новообразований у детей, впервые описанный в 2023 г. Она может локализоваться в любом отделе ЦНС и обусловлена амплификацией генов семейства PLAG. В последнем издании классификации опухолей ЦНС Всемирной организации здравоохранения (2021 г.) данная опухоль как отдельный тип еще не определена. В зарубежной литературе описано 27 случаев эмбриональных опухолей ЦНС с амплификациями PLAGL1 и PLAGL2 у детей. Единого стандарта терапии в настоящее время не разработано, оптимальным способом лечения являются резекция объемного образования, а также полихимиотерапия в сочетании с лучевой терапией.</p><p>В данной статье продемонстрирован клинический случай девочки 2,5 лет с эмбриональной опухолью с амплификацией PLAGL1, подробно описана тактика терапии и представлен анализ случаев, ранее описанных в международной литературе.</p></abstract><trans-abstract xml:lang="en"><p>Embryonal tumor of the central nervous system (CNS) with PLAGL1/PLAGL2 amplification is a new type of CNS tumors in children, first described in 2023. They can be localized in any part of the CNS and, as their name suggests, are caused by the amplification of genes of the PLAG family. At present, this tumor is not yet defined as a separate type in the latest edition of the WHO classification in 2021. In the foreign literature, 27 cases of embryonal tumors of the CNS with PLAGL1 and PLAGL2 amplification in children have been described. A single standard of therapy has not yet been developed; the optimal treatment method is resection of the tumor, as well as polychemotherapy in combination with radiation therapy.</p><p>This article presents a clinical case of a 2.5-year-old girl with an embryonal tumor with PLAGL1 amplification, describes in detail the treatment tactics and presents an analysis of cases previously described in international literature.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>опухоли центральной нервной системы</kwd><kwd>дети</kwd><kwd>эмбриональная опухоль с амплификацией PLAG</kwd></kwd-group><kwd-group xml:lang="en"><kwd>CNS tumors</kwd><kwd>children</kwd><kwd>embryonal tumor with PLAG amplification</kwd></kwd-group><funding-group><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">Wang P., Ma K., Chen T., Xue X., Ma D., Wang S., Chen X., Meng H., Cui G., Gao B., Lin J., Feng H., Chu W. 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