nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.21682/2311-1267-2025-12-3-63-79nodgo-1190Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSГлиомы и глионейрональные опухоли у детей. Интерпретация молекулярных особенностейGliomas and glioneuronal tumors in children. Interpretation of molecular featureshttps://orcid.org/0000-0001-7206-6365РыжоваМ. В.RyzhovaM. V.

Марина Владимировна Рыжова - д.м.н., заведующая патологоанатомическим отделением НМИЦ нейрохирургии им. акад. Н.Н. Бурденко.

125047, Москва, ул. 4-я Тверская-Ямская, 16

Dr. of Sci. (Med.), Head of the Pathology Department.

16 4th Tverskaya-Yamskaya St., Moscow, 125047

mrizhova@nsi.ruhttps://orcid.org/0000-0002-7531-6443ОльховаЛ. В.OlkhovaL. V.

к.м.н., врач-детский онколог отделения трансплантации костного мозга РДКБ – филиала РНИМУ им. Н.И. Пирогова.

119572, Москва, Ленинский просп., 117

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 MHR.

117 Leninskiy Prosp., Moscow, 117997

rylkova87@mail.ruhttps://orcid.org/0000-0002-8607-3635ЖелудковаО. Г.ZheludkovaO. G.

д.м.н., профессор, эксперт ВАК по детской нейроонкологии, главный научный сотрудник НПЦ спец. мед. помощи детям ДЗМ, врач-детский онколог ООО «ЛДЦ МИБС».

119620, Москва, ул. Авиаторов, 38; 194354, Санкт-Петербург, ул. Есенина, 2, корп. 3

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, Diagnostic and Treatment Center of International Institution for Biological Systems named after S. Berezin.

38 Aviatorov St., Moscow, 119620; Bldg. 3, 2 Yesenin St., S.-Petersburg, 194354

clelud@mail.ruhttps://orcid.org/0000-0001-6125-2410КумироваЭ. В.KumirovaE. V.

д.м.н., врач-детский онколог, научный консультант НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова НМИЦ онкологии им. Н.Н. Блохина и РНИМУ им. Н.И. Пирогова, заместитель главного врача по онкологии Морозовской ДГКБ.

117997, Москва, ул. Островитянова, 1; 115522, Москва, Каширское шоссе, 23; 119049, Москва, 4-й Добрынинский пер., 1/9

Dr. of Sci. (Med.), Pediatric Oncologist, Scientific Consultant 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, MHR and N.I. Pirogov Russian National Research Medical University, MHR, Deputy Chief Physician for Oncology Morozov Children’s City Clinical Hospital of the Moscow City Healthcare Department.

1 Ostrovityanova St., Moscow, 117997; 23 Kashirskoe Shosse, Moscow, 115522; 1/9 4th Dobryninskiy Per., Moscow, 119049

k_ella2004@mail.ruhttps://orcid.org/0000-0002-7471-7181БелогуроваМ. Б.BelogurovaM. B.

д.м.н., профессор, ведущий научный сотрудник Института гематологии НМИЦ им. В.А. Алмазова, заведующая отделением химиотерапии и комбинированного лечения опухолей у детей СПб КНПЦСВМП(о) им. Н.П. Напалкова.

197341, Санкт-Петербург, ул. Аккуратова, 2; 197758, Санкт-Петербург, пос. Песочный, ул. Ленинградская, 68А

Dr. of Sci. (Med.), Professor, Leading Scientific Collaborator of Institution of Hematology of Almazov National Medical Research Center, MHR, Head of the Department of Chemotherapy and Combined Treatment of Tumors in Children at Saint-Petersburg Clinical Scientific and Practical Center for Special Types of Medical Care (Oncological) named after N.P. Napalkov.

2 Akkuratova St., S.-Petersburg, 197341; 68А Leningradskaya St., Pesochny, S.-Petersburg, 197758

deton.hospital31@inbox.ruhttps://orcid.org/0000-0002-2003-0982ДиникинаЮ. В.DinikinaYu. V.

д.м.н., заведующая научно-исследовательской лабораторией детской нейроиммуноонкологии Центра персонализированной медицины и заведующая отделением химиотерапии онкогематологических заболеваний и трансплантации костного мозга для детей НМИЦ им. В.А. Алмазова.

197341, Санкт-Петербург, ул. Аккуратова, 2

Dr. 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 for Children at Almazov National Medical Research Center, MHR.

2 Akkuratova St., S.-Petersburg, 197341

dinikina_yuv@almazovcentre.ruhttps://orcid.org/0000-0001-7045-7223ШишкинаЛ. В.ShishkinaL. V.

к.м.н., врач-патологоанатом патологоанатомического отделения НМИЦ нейрохирургии им. акад. Н.Н. Бурденко.

125047, Москва, ул. 4-я Тверская-Ямская, 16

Cand. of Sci. (Med.), Pathologist Pathology Department.

16 4th Tverskaya-Yamskaya St., Moscow, 125047

Lshishkina@nsi.ruhttps://orcid.org/0000-0002-2584-802XСнигиреваГ. П.SnigirevaG. P.

д.б.н., биолог патологоанатомического отделения НМИЦ нейрохирургии им. акад. Н.Н. Бурденко.

125047, Москва, ул. 4-я Тверская-Ямская, 16

Dr. of Sci. (Biol.), Biologist Pathology Department.

16 4th Tverskaya-Yamskaya St., Moscow, 125047

sni_gal@mail.ruhttps://orcid.org/0000-0002-0370-8667ТелышеваЕ. Н.TelyshevaE. N.

к.б.н., биолог патологоанатомического отделения НМИЦ нейрохирургии им. акад. Н.Н. Бурденко.

125047, Москва, ул. 4-я Тверская-Ямская, 16

Cand. of Sci. (Biol.), Biologist Pathology Department.

16 4th Tverskaya-Yamskaya St., Moscow, 125047

telisheva_k@mail.ruФГАУ «Национальный медицинский исследовательский центр нейрохирургии имени академика Н.Н. Бурденко» Минздрава РоссииРоссияN.N. Burdenko National Medical Research Center of Neurosurgery, Ministry of Health of RussiaRussian FederationРоссийская детская клиническая больница – филиал ФГАОУ ВО РНИМУ им. Н.И. Пирогова Минздрава РоссииРоссияRussian Children’s Clinical Hospital – Branch of the N.I. Pirogov Russian National Research Medical University, Ministry of Health of RussiaRussian FederationГБУЗ «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»РоссияV.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 BerezinRussian FederationФГАОУ ВО РНИМУ им. Н.И. Пирогова Минздрава России; ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России; ГБУЗ «Морозовская детская городская клиническая больница Департамента здравоохранения города Москвы»РоссияN.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia; N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia; Morozovskaya Children’s Clinical Hospital of the Moscow City Healthcare DepartmentRussian FederationФГБУ «Национальный медицинский исследовательский центр имени В.А. Алмазова» Минздрава России; ГБУЗ «Санкт-Петербургский клинический научно-практический центр специализированных видов медицинской помощи (онкологический) имени Н.П. Напалкова»РоссияAlmazov National Medical Research Centre, Ministry of Health of Russia; Saint Petersburg Clinical Scientific and Practical Center of Specialized Medical Assistance (Oncological) named after N.P. NapalkovRussian FederationФГБУ «Национальный медицинский исследовательский центр имени В.А. Алмазова» Минздрава РоссииРоссияAlmazov National Medical Research Centre, Ministry of Health of RussiaRussian Federation2025021120251236379Copyright © Рыжова М.В., Ольхова Л.В., Желудкова О.Г., Кумирова Э.В., Белогурова М.Б., Диникина Ю.В., Шишкина Л.В., Снигирева Г.П., Телышева Е.Н., 20252025Рыжова М.В., Ольхова Л.В., Желудкова О.Г., Кумирова Э.В., Белогурова М.Б., Диникина Ю.В., Шишкина Л.В., Снигирева Г.П., Телышева Е.Н.Ryzhova M.V., Olkhova L.V., Zheludkova O.G., Kumirova E.V., Belogurova M.B., Dinikina Y.V., Shishkina L.V., Snigireva G.P., Telysheva E.N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nodgo.org/jour/article/view/1190

В работе собраны данные о генетических нарушениях, наблюдаемых при глиальных и глионейрональных опухолях центральной нервной системы у детей. Часть нарушений могут быть использованы в качестве диагностических маркеров, в то время как выявление других нарушений может быть показанием для назначения таргетной терапии. Для выполнения данной работы авторы объединили свои взгляды диагностов и клиницистов и надеются, что она будет не только способствовать диалогу и пониманию между специалистами, но и, что еще важнее, позволит понять саму классификацию опухолей центральной нервной системы Всемирной организации здравоохранения и, соответственно, облегчит диагностику и лечение таких новообразований.

Кроме того, представленный обзор может быть использован в качестве справочного пособия и практических рекомендаций по диагностике и поиску мишеней для таргетной терапии и будет полезен не только нейроонкологам, но и молекулярным патологам, нейропатологам и биологам. Для удобства читателей часть информации приведена в таблицах.

This review consolidates current knowledge on genetic alterations observed in pediatric glial and glioneuronal tumors of the central nervous system (CNS). Some of these alterations serve as diagnostic biomarkers, whereas others may inform the selection of targeted therapeutic strategies. Drawing on both diagnostic and clinical perspectives, the authors aim to facilitate a more comprehensive understanding of the WHO Classification of CNS Tumors and to promote interdisciplinary collaboration. This resource is intended to support accurate tumor classification, guide molecular testing, and aid in identifying actionable genetic targets. The content may be of practical value to pediatric neuro-oncologists, molecular pathologists, neuropathologists, and researchers involved in CNS tumor diagnostics and treatment. For user convenience, selected data are summarized in tables.

детские глиомыдетские глионейрональные опухолитаргетная терапиямутации геновслияния геновопухоли центральной нервной системыpediatric gliomasglioneuronal tumorstargeted therapygene mutationsgene fusionscentral nervous system tumorsИсследование проведено без спонсорской поддержки. Авторы выражают благодарность Благотворительному фонду Константина Хабенского за поддержку в проведении молекулярных исследований опухолей ЦНС у детей.The study was performed without external fundingReferencesSiegel B.I., Duke E.S., Kilburn L.B., Packer R.J. Molecular-targeted therapy for childhood low-grade glial and glioneuronal tumors. Childs Nerv Syst. 2024;40(10):3251–62. doi: 10.1007/s00381-024-06486-6. Epub 2024 Jun 15. PMID: 38877124.Siegel B.I., Duke E.S., Kilburn L.B., Packer R.J. Molecular-targeted therapy for childhood low-grade glial and glioneuronal tumors. Childs Nerv Syst. 2024;40(10):3251–62. doi: 10.1007/s00381-024-06486-6. Epub 2024 Jun 15. 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Acta Neuropathol. 2021;142(5):827–39. doi: 10.1007/s00401-021-02356-6. Epub 2021 Aug 5. PMID: 34355256; PMCID: PMC8500895.Sievers P., Henneken S.C., Blume C., Sill M., Schrimpf D., Stichel D., Okonechnikov K., Reuss D.E., Benzel J., Maaß K.K., Kool M., Sturm D., Zheng T., Ghasemi D.R., Kohlhof-Meinecke P., Cruz O., Suñol M., Lavarino C., Ruf V., Boldt H.B., Pagès M., Pouget C., Schweizer L., Kranendonk M.E.G., Akhtar N., Bunkowski S., Stadelmann C., Schüller U., Mueller W.C., Dohmen H., Acker T., Harter P.N., Mawrin C., Beschorner R., Brandner S., Snuderl M., Abdullaev Z., Aldape K., Gilbert M.R., Armstrong T.S., Ellison D.W., Capper D., Ichimura K., Reifenberger G., Grundy R.G., Jabado N., Krskova L., Zapotocky M., Vicha A., Varlet P., Wesseling P., Rutkowski S., Korshunov A., Wick W., Pfi ster S.M., Jones D.T.W., von Deimling A., Pajtler K.W., Sahm F. Recurrent fusions in PLAGL1 defi ne a distinct subset of pediatric-type supratentorial neuroepithelial tumors. 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PMID: 33216206; PMCID: PMC7785563.Keck M.K., Sill M., Wittmann A., Joshi P., Stichel D., Beck P., Okonechnikow K., Sievers P., Wefers A.K., Roncaroli F., Avula S., McCabe M.G., Hayden J.T., Wesseling P., Øra I., Nistér M., Kranendonk M.E.G., Tops B.B.J., Zapotocky M., Zamecnik J., Vasiljevic A., Fenouil T., Meyronet D., von Hoff K., Schüller U., Loiseau H., Figarella-Branger D., Kramm C.M., Sturm D., Scheie D., Rauramaa T., Pesola J., Gojo J., Haberler C., Brandner S., Jacques T., Sexton Oates A., Saffery R., Koscielniak E., Baker SJ., Yip S., Snuderl M., Ud Din N., Samuel D., Schramm K., Blattner-Johnson M., Selt F., Ecker J., Milde T., von Deimling A., Korshunov A., Perry A., Pfister S.M., Sahm F., Solomon D.A., Jones D.T.W. Amplification of the PLAG-family genes-PLAGL1 and PLAGL2 – is a key feature of the novel tumor type CNS embryonal tumor with PLAGL amplification. Acta Neuropathol. 2023;145(1):49–69. doi: 10.1007/s00401-022-02516-2. Epub 2022 Nov 27. Erratum in: Acta Neuropathol. 2023;145(4):511–4. doi: 10.1007/s00401-023-02538-4. PMID: 36437415; PMCID: PMC9807491.Keck M.K., Sill M., Wittmann A., Joshi P., Stichel D., Beck P., Okonechnikow K., Sievers P., Wefers A.K., Roncaroli F., Avula S., McCabe M.G., Hayden J.T., Wesseling P., Øra I., Nistér M., Kranendonk M.E.G., Tops B.B.J., Zapotocky M., Zamecnik J., Vasiljevic A., Fenouil T., Meyronet D., von Hoff K., Schüller U., Loiseau H., Figarella-Branger D., Kramm C.M., Sturm D., Scheie D., Rauramaa T., Pesola J., Gojo J., Haberler C., Brandner S., Jacques T., Sexton Oates A., Saff ery R., Koscielniak E., Baker SJ., Yip S., Snuderl M., Ud Din N., Samuel D., Schramm K., Blattner-Johnson M., Selt F., Ecker J., Milde T., von Deimling A., Korshunov A., Perry A., Pfi ster S.M., Sahm F., Solomon D.A., Jones D.T.W. Amplifi cation of the PLAG-family genes-PLAGL1 and PLAGL2 – is a key feature of the novel tumor type CNS embryonal tumor with PLAGL amplifi cation. Acta Neuropathol. 2023;145(1):49–69. doi: 10.1007/s00401-022-02516-2. Epub 2022 Nov 27. Erratum in: Acta Neuropathol. 2023;145(4):511–4. doi: 10.1007/s00401-023-02538-4. PMID: 36437415; PMCID: PMC9807491.Tauziède-Espariat A., Volodia-Dangouloff-Ros, Figarella-Branger D., Uro-Coste E., Nicaise Y., André N., Scavarda D., Testud B., Girard N., Rousseau A., Basset L., Chotard G., Jecko V., le Loarer F., Hostein I., Machet M.C., Tallegas M., Listrat A., Hasty L., Métais A., Chrétien F., Boddaert N., Varlet P.; RENOCLIP-LOC. Clinicopathological and molecular characterization of three cases classified by DNA-methylation profiling as “Glioneuronal Tumors, NOS, Subtype A”. Acta Neuropathol. 2022;144(6):1179–83. doi: 10.1007/s00401-022-02490-9. Epub 2022 Sep 19. 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The authors declare that there are no conflicts of interest present.