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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">10.21682/2311-1267-2024-11-4-51-59</article-id><article-id custom-type="elpub" pub-id-type="custom">nodgo-1097</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>ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ORIGINAL STUDIES</subject></subj-group></article-categories><title-group><article-title>Система раннего прогнозирования прогрессирования саркомы Юинга у детей</article-title><trans-title-group xml:lang="en"><trans-title>System for early prediction of progression of Ewingʼs sarcoma in children</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2310-0106</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Романцова</surname><given-names>О. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Romantsova</surname><given-names>O. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Ольга Михайловна Романцова, врач-детский онколог, заведующая детским онкологическим отделением № 2 (химиотерапии опухолей опорно-двигательного аппарата) НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова</p><p>115522, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Pediatric Oncologist, Head of the Department No. 2 (Chemotherapy of Tumors of the Musculoskeletal System) of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov</p><p>23 Kashirskoe Shosse, Moscow, 115522</p></bio><email xlink:type="simple">dr.roma1986@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-0810-8238</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Прищеп</surname><given-names>П. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Prishchep</surname><given-names>P. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-детский онколог детского онкологического отделения № 2 (химиотерапии опухолей опорно-двигательного аппарата) НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова</p><p>115522, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Pediatric Oncologist Pediatric Oncology Department No. 2 (Chemotherapy of Tumors of the Musculoskeletal System) of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov</p><p>23 Kashirskoe Shosse, Moscow, 115522</p></bio><email xlink:type="simple">Paulig92@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/0000-0003-0883-7801</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Хайруллова</surname><given-names>В. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Khairullova</surname><given-names>V. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-детский онколог детского онкологического отделения № 2 (химиотерапии опухолей опорно-двигательного аппарата) НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова</p><p>115522, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Pediatric Oncologist Pediatric Oncology Department No. 2 (Chemotherapy of Tumors of the Musculoskeletal System) of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov</p><p>23 Kashirskoe Shosse, Moscow, 115522</p></bio><email xlink:type="simple">vini999_999@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/0000-0002-1925-2457</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ефимова</surname><given-names>М. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Efimova</surname><given-names>M. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-детский онколог детского онкологического отделения № 2 (химиотерапии опухолей опорно-двигательного аппарата) НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова</p><p>115522, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Pediatric Oncologist Pediatric Oncology Department No. 2 (Chemotherapy of Tumors of the Musculoskeletal System) of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov</p><p>23 Kashirskoe Shosse, Moscow, 115522</p></bio><email xlink:type="simple">Drefimovaburgol@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/0000-0003-4019-5247</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Валеева</surname><given-names>Е. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Valeeva</surname><given-names>E. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-детский онколог детского онкологического отделения № 2 (химиотерапии опухолей опорно-двигательного аппарата) НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова</p><p>115522, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Pediatric Oncologist Pediatric Oncology Department No. 2 (Chemotherapy of Tumors of the Musculoskeletal System) of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov</p><p>23 Kashirskoe Shosse, Moscow, 115522</p></bio><email xlink:type="simple">liiilerman@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0009-3373-8996</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Алескерова</surname><given-names>Х. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Aleskerova</surname><given-names>Kh. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-детский онколог детского онкологического отделения № 2 (химиотерапии опухолей опорно-двигательного аппарата) НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова</p><p>115522, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Pediatric Oncologist of the Day Hospital with Chemotherapy of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov</p><p>23 Kashirskoe Shosse, Moscow, 115522</p></bio><email xlink:type="simple">khayale97@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/0000-0002-8149-254X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Хестанов</surname><given-names>Д. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Khestanov</surname><given-names>D. B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., старший научный сотрудник детского онкологического отделения № 2 (химиотерапии опухолей опорно-двигательного аппарата) НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова</p><p>115522, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Senior Researcher of the Pediatric Oncology Department No. 2 (Chemotherapy of Tumors of the Musculoskeletal System) of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov</p><p>23 Kashirskoe Shosse, Moscow, 115522</p></bio><email xlink:type="simple">hestanov@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/0000-0002-4807-1516</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Малахова</surname><given-names>А. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Malakhova</surname><given-names>A. А.</given-names></name></name-alternatives><bio xml:lang="ru"><p>заведующая организационно-методическим отделом с канцер-регистром и медицинским архивом НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова</p><p>115522, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Head of the Organizational and Methodological Department with the Cancer Register and the Medical Archive of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov</p><p>23 Kashirskoe Shosse, Moscow, 115522</p></bio><email xlink:type="simple">malalina0506@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/0000-0001-5805-726X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Горбунова</surname><given-names>Т. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Gorbunova</surname><given-names>T. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заместитель главного врача по медицинской части и старший научный сотрудник детского онкологического отделения хирургических методов лечения с проведением химиотерапии № 1 (опухолей головы и шеи) НИИ детской онкологии и гематологии</p><p>115522, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Deputy Chief Physician for Medical Affairs and Senior Researcher Childrenʼs Oncology Department of Surgical Treatment Methods with Chemotherapy No. 1 (Head and Neck Tumors) of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov</p><p>23 Kashirskoe Shosse, Moscow, 115522</p></bio><email xlink:type="simple">wasicsol@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/0000-0002-2945-284X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Киргизов</surname><given-names>К. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Kirgizov</surname><given-names>K. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заместитель директора по научной работе НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова</p><p>115522, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Deputy Director for Scientific Work of Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov</p><p>23 Kashirskoe Shosse, Moscow, 115522</p></bio><email xlink:type="simple">k.kirgizov@ronc.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>20</day><month>01</month><year>2025</year></pub-date><volume>11</volume><issue>4</issue><fpage>51</fpage><lpage>59</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Романцова О.М., Прищеп П.Л., Хайруллова В.В., Ефимова М.М., Валеева Е.В., Алескерова Х.А., Хестанов Д.Б., Малахова А.А., Горбунова Т.В., Киргизов К.И., 2025</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="ru">Романцова О.М., Прищеп П.Л., Хайруллова В.В., Ефимова М.М., Валеева Е.В., Алескерова Х.А., Хестанов Д.Б., Малахова А.А., Горбунова Т.В., Киргизов К.И.</copyright-holder><copyright-holder xml:lang="en">Romantsova O.M., Prishchep P.L., Khairullova V.V., Efimova M.M., Valeeva E.V., Aleskerova K.A., Khestanov D.B., Malakhova A.А., Gorbunova T.V., Kirgizov K.I.</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/1097">https://journal.nodgo.org/jour/article/view/1097</self-uri><abstract><sec><title>Введение</title><p>Введение. Прогноз у больных саркомой Юинга (СЮ) определяет совокупность таких факторов, как размер первичной опухоли, локализация и распространенность процесса, а также клинический ответ на лекарственное лечение. Учет всех прогностических показателей и возможность их корректной оценки необходимы для стратификации риска прогрессирования перед началом лечения и для выбора оптимального подхода в терапии. Нами была спроектирована математическая прогностическая модель, позволяющая сформировать группы риска перед началом программного лечения.</p></sec><sec><title>Материалы и методы</title><p>Материалы и методы. Проведено ретроспективное когортное одноцентровое исследование у детей с СЮ, получавших лечение с 2012 по 2023 г. в НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова ФГБУ ≪НМИЦ онкологии им. Н.Н. Блохина≫ Минздрава России. Для определения значимых независимых факторов прогноза применены однофакторный и многофакторный регрессионный анализ Кокса. Прогностическая значимость оценивалась посредством построения ROC-кривых.</p></sec><sec><title>Результаты</title><p>Результаты. Определены следующие ранее не оцениваемые независимые факторы риска прогрессирования: инициально высокий уровень лактатдегидрогеназы, лейкоцитоз, гиперфибриногенемия и программа лечения. Создана математическая прогностическая модель расчета индивидуального риска прогрессирования. Чувствительность разработанной системы составила 87,7 %, специфичность – 94,0 %, точность – 91,3 %, прогностическая ценность положительного результата – 91,9 %, отрицательного – 90,9 %.</p></sec><sec><title>Выводы</title><p>Выводы. Разработанный индекс индивидуальной вероятности прогрессирования продемонстрировал высокую прогностическую способность у детей с СЮ.</p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Introduction</title><p>Introduction. The prognosis in patients with Ewingʼs sarcoma (ES) is determined by a combination of factors such as the size of the primary tumor, the location and extent of the process, as well as the clinical response to drug treatment. Taking into account all prognostic indicators and the possibility of their correct assessment are necessary to stratify the risk of progression before starting treatment and to select the optimal approach to therapy. We have developed a mathematical prognostic model that allows us to form risk groups before starting program therapy.</p></sec><sec><title>Materials and methods</title><p>Materials and methods. A retrospective cohort single-center study was conducted in children with ES, who received treatment from 2012 to 2023 of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov at N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia. To determine significant independent prognostic factors, univariate and multivariate Cox regression analysis was used. Prognostic significance was assessed by constructing ROC curves.</p></sec><sec><title>Results</title><p>Results. The following previously unassessed independent risk factors for progression were identified: initially high lactate dehydrogenase levels, leukocytosis, hyperfibrinogenemia and treatment program. A mathematical prognostic model for calculating the individual risk of progression has been created. The sensitivity of the developed system was 87.7 %, specificity – 94.0 %, accuracy – 91.3 %, positive predictive value – 91.9%, negative – 90.9 %.</p></sec><sec><title>Conclusions</title><p>Conclusions. The developed Individual Probability of Progression Index demonstrated high prognostic ability in children with ES.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>саркома Юинга</kwd><kwd>система раннего прогноза</kwd><kwd>индекс вероятности прогрессирования</kwd><kwd>общая выживаемость</kwd><kwd>высокоточная математическая модель прогноза</kwd></kwd-group><kwd-group xml:lang="en"><kwd>Ewingʼs sarcoma</kwd><kwd>early prognosis system</kwd><kwd>progression probability index</kwd><kwd>overall survival</kwd><kwd>high-precision mathematical prognosis model</kwd></kwd-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Cotterill S.J., Ahrens S., Paulussen M., Jürgens H.F., Voûte P.A., Gadner H., Craft A.W. Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma Study Group. 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