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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">10.21682/2311-1267-2023-10-1-11-24</article-id><article-id custom-type="elpub" pub-id-type="custom">nodgo-906</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>Сравнительные результаты лечения детей с атипичной тератоидно-рабдоидной опухолью центральной нервной системы в младшей возрастной группе</article-title><trans-title-group xml:lang="en"><trans-title>Comparative treatment results of children with atypical teratoid/rhabdoid tumor of the central nervous system in the younger age group</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>врач-детский онколог отделения трансплантации костного мозга </p><p>119571, Москва, Ленинский просп., 117</p></bio><bio xml:lang="en"><p>Pediatric Oncologist, Department of Bone Marrow Transplantation</p><p>117 Leninskiy Prosp., Moscow, 117997</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, врач-детский онколог </p><p>119620, Москва, ул. Авиаторов, 38</p><p>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</p><p>38 Aviatorov St., Moscow, 119620</p><p>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/0000-0003-2594-7703</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Зубаровская</surname><given-names>Л. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Zubarovskaya</surname><given-names>L. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., заместитель директора по трансплантации, руководитель отдела детской онкологии, гематологии и трансплантологии; профессор кафедры гематологии, трансфузиологии и трансплантологии им. проф. Б.В. Афанасьева </p><p>197022, Санкт-Петербург, ул. Рентгена, 12</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Deputy Director for Transplantation, Head of the Department of Pediatric Oncology, Hematology and Transplantology at Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, Professor for Hematology, Transfusiology and Transplantation Chair named after Professor B.V. Afanasyev</p><p>12 Rentgena St., S.-Petersburg, 197022</p></bio><email xlink:type="simple">zubarovskaya_ls@mail.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/0000-0001-5081-3964</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Левашов</surname><given-names>А. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Levashov</surname><given-names>A. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., старший научный сотрудник детского онкологического отделения хирургических методов лечения с проведением химиотерапии № 1 (опухолей головы и шеи) НИИ ДОиГ 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 at Pediatric Oncology Department of Surgical Treatment with Chemotherapy No. 1 (Head and Neck Tumors) </p><p>23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">andreyslevashov@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/0000-0002-5293-9568</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Смирнова</surname><given-names>А. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Smirnova</surname><given-names>A. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-детский онколог отделения химиотерапии онкогематологических заболеваний и трансплантации костного мозга для детей </p><p>197341, Санкт-Петербург, ул. Аккуратова, 2</p></bio><bio xml:lang="en"><p>Pediatric Oncologist Department of Chemotherapy for Hematologic Diseases and Bone Marrow Transplantation</p><p>2 Akkuratova St., S.-Petersburg, 197341</p></bio><email xlink:type="simple">smirnova_a_yu@almazovcentre.ru</email><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2003-0982</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Диникина</surname><given-names>Ю. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Dinikina</surname><given-names>Yu. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заведующая научно-исследовательской лабораторией детской нейроиммуноонкологии Центра персонализированной медицины и заведующая отделением химиотерапии онкогематологических заболеваний и трансплантации костного мозга для детей</p><p>197341, Санкт-Петербург, ул. Аккуратова, 2</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Head of the Laboratory of Pediatric Neuro-Immuno-Oncology of the Pesonalized Medicine Centre and Head of the Department of Chemotherapy for Hematologic Diseases and Bone Marrow Transplantation </p><p>2 Akkuratova St., S.-Petersburg, 197341</p></bio><email xlink:type="simple">dinikina_yuv@almazovcentre.ru</email><xref ref-type="aff" rid="aff-5"/></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 </p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Professor, Neurosurgeon of the 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-6"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-0737-5983</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Меликян</surname><given-names>А. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Melikyan</surname><given-names>A. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., профессор, заведующий 2-м нейрохирургическим отделением</p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Professor, Head of the 2nd Children Neurosurgical Department</p><p>16 4th Tverskaya-Yamskaya St., Moscow, 125047</p></bio><email xlink:type="simple">Melikian@nsi.ru</email><xref ref-type="aff" rid="aff-6"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-0984-2039</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Горелышев</surname><given-names>С. К.</given-names></name><name name-style="western" xml:lang="en"><surname>Gorelyshev</surname><given-names>S. K.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., профессор, заведующий 1-м детским нейрохирургическим отделением</p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Professor, Head of the 1st Children Neurosurgical Department</p><p>16 4th Tverskaya-Yamskaya St., Moscow, 125047</p></bio><email xlink:type="simple">sgorel@nsi.ru</email><xref ref-type="aff" rid="aff-6"/></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</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-6"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-4240-5036</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Трунин</surname><given-names>Ю. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Trunin</surname><given-names>Yu. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., старший научный сотрудник</p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Senior Researcher</p><p>16 4th Tverskaya-Yamskaya St., Moscow, 125047</p></bio><email xlink:type="simple">ytrunin@nsi.ru</email><xref ref-type="aff" rid="aff-6"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2905-8209</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Геворгян</surname><given-names>А. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Gevorgyan</surname><given-names>A. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заведующая отделением трансплантации костного мозга для детей № 2; доцент кафедры гематологии, трансфузиологии и трансплантологии им. проф. Б.В. Афанасьева </p><p>197022, Санкт-Петербург, ул. Рентгена, 12</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Head of the 2nd Pediatric Transplant Department of Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, Assistant Professor for Hematology, Transfusiology and Transplantation Chair named after Professor B.V. Afanasyev </p><p>12 Rentgena St., S.-Petersburg, 197022</p></bio><email xlink:type="simple">asmikgevorgian@gmail.com</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9364-5881</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Полушкина</surname><given-names>О. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Polushkina</surname><given-names>O. B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заведующая онкологическим отделением № 2</p><p>119620, Москва, ул. Авиаторов, 38</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Head of the 2nd Oncology Department</p><p>38 Aviatorov St., Moscow, 119620</p></bio><email xlink:type="simple">opolushkina@mail.ru</email><xref ref-type="aff" rid="aff-7"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4835-8047</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Попов</surname><given-names>В. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Popov</surname><given-names>V. E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., врач-нейрохирург</p><p>129110, Москва, ул. Щепкина 61/2, корп. 1;</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Neurosurgeon </p><p> 61/2 Shchepkina St., Moscow, 129110</p></bio><email xlink:type="simple">1popov1@list.ru</email><xref ref-type="aff" rid="aff-8"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Привалова</surname><given-names>Л. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Privalova</surname><given-names>L. P.</given-names></name></name-alternatives><bio xml:lang="ru"><p>заведующая онкологическим отделением</p><p>603136, Нижний Новгород, ул. Ванеева, 211</p></bio><bio xml:lang="en"><p>Head of Oncology Department</p><p>211 Vaneeva St., Nizhny Novgorod, 603136</p></bio><email xlink:type="simple">doprival@mail.ru</email><xref ref-type="aff" rid="aff-9"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-7305-6959</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Юдина</surname><given-names>Н. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Yudina</surname><given-names>N. B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-гематолог, заведующая онкогематологическим отделением; главный внештатный детский специалист онколог-гематолог Воронежской области</p><p>394087, Воронеж, ул. Ломоносова, 114</p></bio><bio xml:lang="en"><p>Hematologist, Head of the Oncohematology Department</p><p>114 Lomonosova St., Voronezh, 394087</p></bio><email xlink:type="simple">n.yudina@list.ru</email><xref ref-type="aff" rid="aff-10"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9331-5049</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Погорелов</surname><given-names>Д. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Pogorelov</surname><given-names>D. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>заведующий онкологическим отделением </p><p>398042, Липецк, ул. Московская, 6а</p></bio><bio xml:lang="en"><p>Head of Oncology Department Children’s Clinical Hospital</p><p>6a Moskovskaya St., Lipetsk, 398042</p></bio><email xlink:type="simple">rylkova87@mail.ru</email><xref ref-type="aff" rid="aff-11"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3327-3772</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Горбатых</surname><given-names>С. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Gorbatykh</surname><given-names>S. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-детский онколог отделения детской онкологии </p><p>119049, Москва, 4-й Добрынинский пер., 1/9</p></bio><bio xml:lang="en"><p>Pediatric Oncologist of the Oncology Department</p><p>1/9 4th Dobryninskiy Per., Moscow, 119049</p></bio><email xlink:type="simple">svetalotos-07@gmail.com</email><xref ref-type="aff" rid="aff-12"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6998-5771</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Воробьёв</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Vorobyov</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>194354, Санкт-Петербург, ул. Есенина, 2, корп. 3</p><p>199034, Санкт-Петербург, Университетская наб., 7/9</p><p>191015, Санкт-Петербург, ул. Кирочная, 41</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Radiation Therapist, Head of the Department Proton Radiotherapy, Assistant Professor Department of Oncology</p><p>Bldg. 3, 2 Yesenin St., S.-Petersburg, 194354</p><p>7/9 Universitetskaya Emb., S.-Petersburg, 199034</p><p>41 Kirochnaya St., S.-Petersburg, 191051</p></bio><email xlink:type="simple">vorobyov@ldc.ru</email><xref ref-type="aff" rid="aff-13"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-9131-7924</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Плахотина</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Plakhotina</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., врач-рентгенолог ЛДЦ МИБС</p><p>194354, Санкт-Петербург, ул. Есенина, 2, корп. 3</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Radiologist</p><p>Bldg. 3, 2 Yesenin St., S.-Petersburg, 194354</p></bio><email xlink:type="simple">plahotina@ldc.ru</email><xref ref-type="aff" rid="aff-14"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1679-5173</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Мартынова</surname><given-names>Н. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Martynova</surname><given-names>N. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-радиотерапевт отделения протонной терапии</p><p>194354, Санкт-Петербург, ул. Есенина, 2, корп. 3</p></bio><bio xml:lang="en"><p>Radiation Therapist Clinics Radiosurgery, Stereotactic Radiotherapy and General Oncology</p><p>Bldg. 3, 2 Yesenin St., S.-Petersburg, 194354</p></bio><email xlink:type="simple">martynova@ldc.ru</email><xref ref-type="aff" rid="aff-14"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7504-5973</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Скворцова</surname><given-names>Т. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Skvortcova</surname><given-names>T. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., ведущий научный сотрудник лаборатории нейровизуализации</p><p>197376, Санкт-Петербург, ул. Академика Павлова, 9</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Senior Research Associate Department of Neurovisualisation </p><p>9 Academic Pavlov St., S.-Petersburg, 197376</p></bio><email xlink:type="simple">tanya@ihb.spb.ru</email><xref ref-type="aff" rid="aff-15"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-0462-6591</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Зайчиков</surname><given-names>А. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Zaychikov</surname><given-names>A. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>заведующий онкологическим отделением</p><p>620149, Екатеринбург, ул. Серафимы Дерябиной, 32</p></bio><bio xml:lang="en"><p>Head of Oncology Department</p><p>32 Seraphimy Deryabinoy St., Yekaterinburg, 620149</p></bio><xref ref-type="aff" rid="aff-16"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-5093-9196</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Мушинская</surname><given-names>М. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Mushinskaya</surname><given-names>M. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., врач-детский онколог, гематолог </p><p>614066, Пермь, ул. Баумана, 22</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Pediatric Oncologist, Hematologist </p><p>22, Baumana St., Perm, 614066</p></bio><email xlink:type="simple">marina.mushinskaya08@mail.ru</email><xref ref-type="aff" rid="aff-17"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2663-068X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сакун</surname><given-names>Д. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Sakun</surname><given-names>D. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-детский онколог отделения детской онкологии и гематологии с химиотерапией</p><p>270004, Симферополь, ул. Титова, 71</p></bio><bio xml:lang="en"><p>Pediatric Oncologist </p><p>71 Titova St., Simferopol, 270004</p><p> </p></bio><email xlink:type="simple">hematology@child-hosp.ru</email><xref ref-type="aff" rid="aff-18"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4637-1707</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Минкина</surname><given-names>Л. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Minkina</surname><given-names>L. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заведующая онкологическим отделением </p><p>690078, Приморский край, Владивосток, просп. Острякова, 27</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Head of Oncology Department</p><p>27 Ostryakova Prosp., Vladivostok, Primorsky Krai, 690078</p></bio><xref ref-type="aff" rid="aff-19"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2079-1482</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Щепкина</surname><given-names>Е. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Shchepkina</surname><given-names>E. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.соц.н., директор по статистике Департамента «Статистика для медицины», заместитель начальника отдела статистики и сводного контингента Учебно-методического управления </p><p>141407, Химки, ул. Германа Титова, 2</p><p>119571, Москва, просп. Вернадского, 82, стр. 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Soc.), Head of Department Medical Statistics, Deputy Head of the Department of Statistics and the Consolidated Contingent of the Educational and Methodological Department </p><p>2 Hermana Titova St., Khimki, 141407</p><p>Bldg. 1, 82 Vernadsky Prosp., Moscow, 119571</p></bio><email xlink:type="simple">elenaschepkina@gmail.com</email><xref ref-type="aff" rid="aff-20"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Российская детская клиническая больница ФГАОУ ВО «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Russian Children’s Clinical Hospital 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 «Научно-практический центр специализированной медицинской помощи детям имени В.Ф. Войно-Ясенецкого Департамента здравоохранения города Москвы»; ООО «Лечебно-диагностический центр Международного института биологических систем имени Сергея Березина»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Scientific and Practical Center for Specialized Medical Care for Children named after V.F. Voyno-Yasenetsky Moscow Healthcare Department; 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>Научно-исследовательский институт детской онкологии, гематологии и трансплантологии имени Р.М. Горбачевой ФГБОУ ВО «Первый Санкт-Петербургский государственный медицинский университет имени академика И.П. Павлова»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, First Pavlov State Medical University of St. Petersburg, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>ФГБУ «Национальный медицинский исследовательский центр онкологии имени 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><aff-alternatives id="aff-5"><aff xml:lang="ru"><institution>ФГБУ «Национальный медицинский исследовательский центр имени В.А. Алмазова» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Almazov National Medical Research Centre, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-6"><aff xml:lang="ru"><institution>ФГАУ «Национальный медицинский исследовательский центр нейрохирургии имени академика 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-7"><aff xml:lang="ru"><institution>ГБУЗ «Научно-практический центр специализированной медицинской помощи детям имени В.Ф. Войно-Ясенецкого Департамента здравоохранения города Москвы»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Scientific and Practical Center for Specialized Medical Care for Children named after V.F. Voyno-Yasenetsky Moscow Healthcare Department</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-8"><aff xml:lang="ru"><institution>ГБУЗ МО «Московский областной научно-исследовательский клинический институт имени М.Ф. Владимирского»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Moscow Regional Research and Clinical Institute (“MONIKI”)</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-9"><aff xml:lang="ru"><institution>ГБУЗ НО «Нижегородская областная детская клиническая больница»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Nizhny Novgorod Regional Children’s Clinical Hospital</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-10"><aff xml:lang="ru"><institution>БУЗ ВО «Воронежская областная детская клиническая больница № 1»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Voronezh Regional Children’s Clinical Hospital No 1</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-11"><aff xml:lang="ru"><institution>ГУЗ «Областная детская больница»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Lipetsk Regional Children’s Hospital</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-12"><aff xml:lang="ru"><institution>ГБУЗ «Морозовская детская городская клиническая больница Департамента здравоохранения города Москвы»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Morozovskaya Children’s Clinical Hospital, Department of Health in Moscow</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-13"><aff xml:lang="ru"><institution>ООО «Лечебно-диагностический центр Международного института биологических систем имени Сергея Березина»; ФГБОУ ВО «Санкт-Петербургский государственный университет»; ФГБОУ ВО «Северо-Западный государственный медицинский университет имени И.И. Мечникова» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Diagnostic and Treatment Center of International Institution for Biological Systems named after Sergey Berezin; Saint-Petersburg State University; North-Western State Medical University named after I.I. Mechnikov, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-14"><aff xml:lang="ru"><institution>ООО «Лечебно-диагностический центр Международного института биологических систем имени Сергея Березина»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>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-15"><aff xml:lang="ru"><institution>ФГБУН Институт мозга человека имени Н.П. Бехтеревой Российской академии наук</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.P. Bekhtereva Institute of the Human Brain, Russian Academy of Sciences</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-16"><aff xml:lang="ru"><institution>ГАУЗ СО «Областная детская клиническая больница»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Regional Children’s Clinical Hospital</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-17"><aff xml:lang="ru"><institution>Онкогематологический центр имени Ф.П. Гааза ГБУЗ ПК «Краевая детская клиническая больница»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>F.P. Haass Center of Pediatric Oncology and Hematology, Regional Children’s Clinical Hospital</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-18"><aff xml:lang="ru"><institution>ГБУЗ РК «Республиканская детская клиническая больница»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Crimean Republican Pediatric Clinical Hospital</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-19"><aff xml:lang="ru"><institution>ГБУЗ «Краевая детская клиническая больница № 1»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Regional Children’s Clinical Hospital No 1</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-20"><aff xml:lang="ru"><institution>ООО «Техдепартамент»; ФГБОУ ВО «Российская академия народного хозяйства и государственной службы при Президенте Российской Федерации»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>LLC Techdepartament; Russian Presidential Academy of National Economy and Public Administration</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2023</year></pub-date><pub-date pub-type="epub"><day>17</day><month>04</month><year>2023</year></pub-date><volume>10</volume><issue>1</issue><elocation-id>11–24</elocation-id><permissions><copyright-statement>Copyright &amp;#x00A9; Ольхова Л.В., Желудкова О.Г., Зубаровская Л.С., Левашов А.С., Смирнова А.Ю., Диникина Ю.В., Кушель Ю.В., Меликян А.Г., Горелышев С.К., Рыжова М.В., Трунин Ю.Ю., Геворгян А.Г., Полушкина О.Б., Попов В.Е., Привалова Л.П., Юдина Н.Б., Погорелов Д.Н., Горбатых С.В., Воробьёв Н.А., Плахотина Н.А., Мартынова Н.И., Скворцова Т.Ю., Зайчиков А.Н., Мушинская М.В., Сакун Д.Л., Минкина Л.М., Щепкина Е.В., 2023</copyright-statement><copyright-year>2023</copyright-year><copyright-holder xml:lang="ru">Ольхова Л.В., Желудкова О.Г., Зубаровская Л.С., Левашов А.С., Смирнова А.Ю., Диникина Ю.В., Кушель Ю.В., Меликян А.Г., Горелышев С.К., Рыжова М.В., Трунин Ю.Ю., Геворгян А.Г., Полушкина О.Б., Попов В.Е., Привалова Л.П., Юдина Н.Б., Погорелов Д.Н., Горбатых С.В., Воробьёв Н.А., Плахотина Н.А., Мартынова Н.И., Скворцова Т.Ю., Зайчиков А.Н., Мушинская М.В., Сакун Д.Л., Минкина Л.М., Щепкина Е.В.</copyright-holder><copyright-holder xml:lang="en">Olkhova L.V., Zheludkova O.G., Zubarovskaya L.S., Levashov A.S., Smirnova A.Y., Dinikina Y.V., Kushel Y.V., Melikyan A.G., Gorelyshev S.K., Ryzhova M.V., Trunin Y.Y., Gevorgyan A.G., Polushkina O.B., Popov V.E., Privalova L.P., Yudina N.B., Pogorelov D.N., Gorbatykh S.V., Vorobyov N.A., Plakhotina N.A., Martynova N.I., Skvortcova T.Y., Zaychikov A.N., Mushinskaya M.V., Sakun D.L., Minkina L.M., Shchepkina E.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/906">https://journal.nodgo.org/jour/article/view/906</self-uri><abstract><sec><title>Введение</title><p>Введение. Атипичная тератоидно-рабдоидная опухоль (АТРО) центральной нервной системы (ЦНС) относится к группе эмбриональных новообразований, встречается преимущественно у детей в возрасте до 3 лет, характеризуется крайне агрессивным клиническим течением и неблагоприятным прогнозом. Однако недостаточно данных в отношении разнородности клинического течения АТРО ЦНС и роли клинических и терапевтических прогностических факторов у пациентов в возрасте до 1 года и 2–3 лет.</p><p>Целью данного исследования было проведение сравнительной оценки результатов лечения детей с АТРО ЦНС в указанных возрастных группах.</p></sec><sec><title>Материалы и методы</title><p>Материалы и методы. С 2008 по 2021 г. в исследование были включены 106 пациентов, которым проводилось программное лечение по различным протоколам (АТРО-2006, MUV-ATRT, EU-RHAB и индивидуальные схемы терапии).</p><p>В зависимости от возраста пациенты были разделены на 2 группы:  от 1 до 12 месяцев – 41 пациент, от 13 до 36 месяцев – 65 пациентов. Медиана возраста составила 16 (9–23) месяцев. Указанные группы больных (1–12 месяцев и 13–36 месяцев) статистически значимо не различались по клиническим и терапевтическим характеристикам (пол, первичная локализация и распространенность опухолевого поражения, стадия заболевания, объем удаления первичного опухолевого очага, протоколы лечения, доля пациентов, получивших лучевую терапию (ЛТ), высокодозную полихимиотерапию (ВДПХТ) с аутологичной трансплантацией гемопоэтических стволовых клеток (ауто-ТГСК), интратекальную/интравентрикулярную химиотерапию).</p></sec><sec><title>Результаты</title><p>Результаты. На момент анализа живы 48 (45,3 %) пациентов, умерли 58 (54,7 %) больных, из них 52 (90 %) от прогрессирования болезни и 6 (10 %) от осложнений полихимиотерапии (ПХТ). Показатель однолетней выживаемости без прогрессирования (ВБП) в группе от 1 до 12 месяцев составил 32 %, 2-летней – 18 %, 5-летней – 18 %; показатель однолетней общей выживаемости (ОВ) – 53 %, 2-летней – 29 %, 5-летней – 25 % при медиане наблюдения 8 и 13 мес соответственно. Показатель однолетней ВБП в группе от 13 до 36 месяцев составил 61 %, 2-летней – 36 %, 5-летней – 33 %; показатель однолетней ОВ был равен 86 %, 2-летней – 67 %, 5-летней – 49 % при медиане наблюдения 19 и 38 мес соответственно. В нашем исследовании были определены основные предикторы улучшения показателей ВБП и ОВ у пациентов с АТРО ЦНС в каждой возрастной группе. Для пациентов в возрасте 1–12 месяцев факторами, улучшающими выживаемость, являлись отсутствие метастатических очагов поражения на момент постановки диагноза, проведение терапии по протоколу MUV-ATRT, ЛТ и ВДПХТ с ауто-ТГСК, а в группе 13–36 месяцев – тотальная резекция первичного опухолевого очага, проведение стандартной ПХТ по протоколу АТРО-2006 и ЛТ.</p></sec><sec><title>Заключение</title><p>Заключение. Несмотря на то, что наличие диагноза АТРО ЦНС у детей младше 3 лет обычно ассоциируется с высоким риском развития прогрессирования и рецидива заболевания, результаты нашего исследования демонстрируют неоднородность клинического и терапевтического профилей в указанной возрастной группе пациентов.</p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Introduction</title><p>Introduction. Atypical teratoid/rhabdoid tumor (ATRT) of the central nervous system (CNS) belongs to the embryonic group, occurs mainly in children under 3 years of age and is characterized by an extremely aggressive clinical course and unfavorable outcome. However, there is not enough data about the heterogeneity of the clinical course of CNS ATRT and the role of clinical and therapeutic prognostic factors in patients under 1 year of age and 1–3 years of age.</p><p>The aim of this study was to conduct a comparative evaluation of treatment results in children with CNS ATRT in the indicated age groups.</p></sec><sec><title>Materials and methods</title><p>Materials and methods. From 2008 till 2021 years 106 patients were included in this study. Each patient underwent a therapeutic program according to determined protocol (ATRT-2006, MUV-ATRT, EU-RHAB and individual variant). All patients were divided into 2 groups according to age: from 1 to 12 months – 41 patients, from 13 to 36 months – 65. Median of the age was 16 (9–23) months. These cohorts of patients (1–12 months and 13–36 months) were comparable in clinical and therapeutic characteristics depending on gender, localization of the primary tumor site, stage of the disease, extent of surgical resection, therapeutic protocol, number of patients, who underwent radiation therapy (RT), high-dose chemotherapy (HDCT) with autologous hematopoietic stem cell transplantation (auto-HSCT), intrathecal/ intraventricular chemotherapy.</p></sec><sec><title>Results</title><p>Results. At the time of analysis 48 (45.3 %) patients were alive, 58 (54.7 %) patients died, of whom 52 (90 %) from disease progression and 6 (10 %) from therapeutic complications. One-year progression-free survival (PFS) in the group from 1 to 12 months was 32 %, 2-year – 18 %, 5-year – 18 %; one-year overall survival (OS) – 53 %, 2-year – 29 %, 5-year – 25 % with a median follow-up 8 and 13 months respectively. One-year PFS in the group from 13 to 36 months was 61 %, 2-year – 36 %, 5-year – 33 %; one-year OS – 86 %, 2-year – 67 %, 5-year – 49 % with a median follow-up 19 and 38 months respectively. In our study we identified the main predictors of the PFS and OS improvement in patients with ATRT CNS of each age group. In the 1–12 month group, these factors were: absence of metastatic involvement, MUV-ATRT therapeutic protocol, RT and HDCT with auto-HSCT; in the 13–36 months group: gross total/near gross total resection of the primary tumor cite, ATRT-2006 therapeutic protocol and RT.</p></sec><sec><title>Conclusion</title><p>Conclusion. Despite the fact, that the presence of a diagnosis of ATRT CNS in children under 3 years of age is usually associated with a high risk of disease progression and recurrence, the results of our study demonstrate the heterogeneity of the clinical and therapeutic profile in this age group of patients.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>дети</kwd><kwd>атипичная тератоидно-рабдоидная опухоль центральной нервной системы</kwd><kwd>химиотерапия</kwd><kwd>лучевая терапия</kwd><kwd>результаты лечения</kwd><kwd>выживаемость</kwd><kwd>прогностические факторы</kwd></kwd-group><kwd-group xml:lang="en"><kwd>children</kwd><kwd>atypical teratoid/rhabdoid tumor of the central nervous system</kwd><kwd>chemotherapy</kwd><kwd>radiation therapy</kwd><kwd>treatment results</kwd><kwd>survival</kwd><kwd>prognostic factors</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">Louis D.N., Perry A., Reifenberger G., von Deimling A., Figarella-Branger D., Cavenee W.K., Ohgaki H., Wiestler O.D., Kleihues P., Ellison D.W. 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