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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">10.21682/2311-1267-2025-12-2-96-106</article-id><article-id custom-type="elpub" pub-id-type="custom">nodgo-1155</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>Рецидивы нейробластомы группы высокого риска с изолированным лептоменингеальным поражением</article-title><trans-title-group xml:lang="en"><trans-title>Isolated leptomeningeal relapses in high-risk neuroblastoma</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0004-5751-2063</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Турчина</surname><given-names>А. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Turchina</surname><given-names>A. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Врач-ординатор детский онколог, старший лаборант кафедры онкологии, гематологии и лучевой терапии Института материнства и детства РНИМУ им. Н.И. Пирогова.</p><p>117997, Москва, ул. Островитянова, 1</p></bio><bio xml:lang="en"><p>Resident, Pediatric Oncologist, Senior Laboratory Assistant Department of Oncology, Hematology and Radiotherapy of the Institute of Motherhood and Childhood N.I. Pirogov Russian National Research Medical University, Ministry of Health of the Russia.</p><p>1 Ostrovityanova St., Moscow, 117997</p></bio><email xlink:type="simple">doc@aturchina.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 at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia.</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-2"/></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 at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">andreeva793@bk.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/0000-0001-7479-0007</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Уталиева</surname><given-names>Д. Т.</given-names></name><name name-style="western" xml:lang="en"><surname>Utalieva</surname><given-names>D. T.</given-names></name></name-alternatives><bio xml:lang="ru"><p>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 Department of Clinical Oncology at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">dinara.utalieva@dgoi.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/0000-0002-9654-084X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Моисеенко</surname><given-names>Р. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Moiseenko</surname><given-names>R. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>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 Department of Clinical Oncology at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">doctor-moiseenko@yandex.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/0000-0001-8578-6572</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сидоров</surname><given-names>И. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Sidorov</surname><given-names>I. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., врач-патологоанатом патологоанатомического отделения НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Pathologist Pathological Anatomical Department at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology, Ministry of Health of Russia.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">ilya93sidorov@gmail.com</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7732-8184</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 патологоанатомическим отделением 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 Pathological Anatomical Department at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology, Ministry of Health of Russia.</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-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-8228-4876</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Дубровина</surname><given-names>М. Э.</given-names></name><name name-style="western" xml:lang="en"><surname>Dubrovina</surname><given-names>M. E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Биолог клинико-диагностической лаборатории НМИЦ ДГОИ им. Дмитрия Рогачева.</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Biologist of the Clinical and Diagnostic Laboratory at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">maria.dubrovina@dgoi.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/0000-0002-0521-9277</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Гвоздев</surname><given-names>А. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Gvozdev</surname><given-names>A. A.</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 Radiological Department at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia.</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">gvzalex@yandex.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/0000-0002-3767-4477</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>Татьяна Викторовна Шаманская - 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 at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia.</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-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГАОУ ВО РНИМУ им. Н.И. Пирогова Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>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>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>01</day><month>08</month><year>2025</year></pub-date><volume>12</volume><issue>2</issue><fpage>96</fpage><lpage>106</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Турчина А.Н., Качанов Д.Ю., Андреева Н.А., Уталиева Д.Т., Моисеенко Р.А., Сидоров И.В., Коновалов Д.М., Дубровина М.Э., Гвоздев А.А., Шаманская Т.В., 2025</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="ru">Турчина А.Н., Качанов Д.Ю., Андреева Н.А., Уталиева Д.Т., Моисеенко Р.А., Сидоров И.В., Коновалов Д.М., Дубровина М.Э., Гвоздев А.А., Шаманская Т.В.</copyright-holder><copyright-holder xml:lang="en">Turchina A.N., Kachanov D.Y., Andreeva N.A., Utalieva D.T., Moiseenko R.A., Sidorov I.V., Konovalov D.M., Dubrovina M.E., Gvozdev A.A., Shamanskaya 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/1155">https://journal.nodgo.org/jour/article/view/1155</self-uri><abstract><sec><title>Актуальность</title><p>Актуальность. Нейробластома (НБ) – это наиболее распространенная экстракраниальная злокачественная солидная опухоль детского возраста, около 50 % больных на момент постановки диагноза стратифицируются в группу высокого риска (ВР). Несмотря на мультимодальный подход к терапии, у половины пациентов развивается рецидив заболевания с преимущественным поражением костей скелета и костного мозга. Рецидивы с локализацией в центральной нервной системе (ЦНС) встречаются крайне редко и носят преимущественно паренхиматозный характер. Изолированное лептоменингеальное поражение представляет собой невероятно редкое (0,6 % всех рецидивов), но крайне агрессивное проявление рецидива НБ, ассоциированное с неблагоприятным прогнозом.</p></sec><sec><title>Материалы и методы</title><p>Материалы и методы. Представлено описание 3 клинических случаев развития ЦНС-рецидивов с изолированным лептоменингеальным поражением у пациентов с НБ группы ВР, получавших лечение в НМИЦ ДГОИ им. Дмитрия Рогачева.</p></sec><sec><title>Результаты</title><p>Результаты. В исследование включены 3 пациента с изолированным лептоменингеальным рецидивом (ЛМР) НБ. Их возраст на момент постановки первичного диагноза составил 23, 36 и 80 месяцев. Инициально больные были стратифицированы в группу ВР согласно протоколу GPOH NB-2004. У 2 пациентов была обнаружена амплификация в гене MYCN, у 1 больного – увеличение числа копий (gain) гена MYCN; также у 2 детей была выявлена делеция хромосомного региона 1p, у одного – делеция хромосомного региона 11q. У 2 пациентов обнаружена мутация в гене ALK: в одном случае – в ткани первичной опухоли, в другом – в ткани рецидивной опухоли. Время от постановки первичного диагноза до констатации ЛМР заболевания составило 5, 15 и 16 мес. В 2 случаях ЦНС-рецидив дебютировал с появления клинической симптоматики, в 1 наблюдении он был выявлен при проведении контрольного обследования. Все пациенты получали противорецидивную терапию препаратами, проникающими через гематоэнцефалический барьер, двое – молекулярно-направленную терапию ингибиторами ALK, только 1 больной получил краниоспинальное облучение и интратекальное введение химиопрепарата.</p><p>Двое детей умерли на фоне прогрессирования заболевания (ПЗ). Один пациент на момент публикации жив без ПЗ, время наблюдения составляет 9 мес от констатации рецидива.</p></sec><sec><title>Выводы</title><p>Выводы. Лептоменингеальное поражение ЦНС в рецидиве НБ может характеризоваться неспецифической клинической презентацией, требующей дифференциального диагноза с токсичностью терапии. Необходим комплексный терапевтический подход с проведением химиотерапии, лучевой терапии, интратекального введения препаратов для улучшения отдаленной выживаемости этой подгруппы больных.</p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Background</title><p>Background. Neuroblastoma (NB) is the most common extracranial solid malignant tumor of childhood, about 50 % of patients with NB are stratified into a high-risk (HR) group at the time of diagnosis. Despite the multimodal approach to therapy, half of the patients develop relapse of the disease, affecting mostly bones and bone marrow. Relapses with localization in the central nervous system (CNS) are extremely rare and are predominantly parenchymal in nature. Isolated leptomeningial involvement at relapse is an incredibly rare (0.6 % of all relapses), but is characterized by extremely aggressive manifestation and associated with unfavorable prognosis.</p></sec><sec><title>Materials and methods</title><p>Materials and methods. A description of three clinical cases of CNS relapses with isolated leptomeningeal lesions in patients with HR NB treated at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia.</p></sec><sec><title>Results</title><p>Results. The study included 3 patients with isolated leptomeningeal relapse of NB. The age at the time of the initial diagnosis was 23, 36, and 80 months. Initially, the patients were stratified into a HR group according to the GPOH NB-2004 protocol. Amplification in the MYCN gene was detected in two patients, gain MYCN in one; deletion of the 1p chromosomal region was also detected in two patients, and deletion of the 11q chromosomal region in one patient. Two patients had a mutation in the ALK gene: in one case, the mutation was found in the primary tumor tissue, in the other – in the relapse tumor tissue. The time from the initial diagnosis to the detection of leptomeningeal recurrence of the disease was 5, 15 and 16 months. In two cases, CNS relapse debuted with the appearance of clinical symptoms, in one case it was detected during a follow-up examination. All patients received anti-relapse therapy with drugs that penetrate the blood-brain barrier, two received molecular-directed therapy with ALK inhibitors, and only one patient received craniospinal irradiation and intrathecal administration of a chemotherapy drug.</p><p>Two patients died as the disease progressed. One patient is alive at the time of the publication without disease progression, the follow-up time is 9 months from the diagnosis of relapse.</p></sec><sec><title>Conclusions</title><p>Conclusions. Leptomenigial CNS lesion in relapse NB may be characterized by a non-specific clinical presentation requiring a differential diagnosis with the toxicity of therapy. Comprehensive therapeutic approach with chemotherapy, radiation therapy, and intrathecal administration of drugs is needed to improve the long-term survival of this subgroup of patients.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>нейробластома</kwd><kwd>дети</kwd><kwd>группа высокого риска</kwd><kwd>рецидивы</kwd><kwd>лептоменингеальное поражение</kwd></kwd-group><kwd-group xml:lang="en"><kwd>neuroblastoma</kwd><kwd>children</kwd><kwd>high-risk group</kwd><kwd>relapses</kwd><kwd>leptomeningeal involvement</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">Шаманская Т.В., Качанов Д.Ю., Ликарь Ю.Н., Коновалов Д.М., Дубровина М.Э., Щербаков А.П., Друй А.Е., Андреева Н.А., Шарлай А.С. 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