nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.21682/2311-1267-2020-7-1-41-50nodgo-573Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSАтипические тератоидно-рабдоидные опухоли: молекулярно-генетические особенности, перспективы лечения. Обзор литературыAtypical teratoid-rhabdoid tumors: molecular genetics feutures, perspectives of treatment. Review of literaturehttps://orcid.org/0000-0002-5293-9568СмирноваА. Ю.SmirnovaA. Yu.

врач-детский онколог отделения химиотерапии онкогематологических заболеваний и трансплантации костного мозга для детей НМИЦ им. В.А. Алмазова

197341, Санкт-Петербург, ул. Аккуратова, 2

Pediatric Oncologist Department of Chemotherapy for Hematologic Diseases and Bone Marrow Transplantation for Children

2 Akkuratova St., S.-Petersburg, 197341, Russia

misha12_09@mail.ruhttps://orcid.org/0000-0002-7902-5520ГончаровА. Ю.GoncharovA. Yu.

клинический ординатор по специальности «нейрохирургия» НМИЦ им. В.А. Алмазова

197341, Санкт-Петербург, ул. Аккуратова, 2

Clinical Resident in the specialty “Neurosurgery”

2 Akkuratova St., S.-Petersburg, 197341, Russia

mr.goncharov1994@yandex.ruhttps://orcid.org/0000-0002-2003-0982ДиникинаЮ. В.DinikinaYu. V.

к.м.н., заведующая отделением химиотерапии онкогематологических заболеваний и трансплантации костного мозга для детей НМИЦ им. В.А. Алмазова; доцент кафедры онкологии, детской онкологии и лучевой терапии СПбГПМУ

197341, Санкт-Петербург, ул. Аккуратова, 2; 194100, Санкт-Петербург, ул. Литовская, 2

Cand. of Sci. (Med.), Head of the Department of Chemotherapy for Hematologic Diseases and Bone Marrow Transplantation for Children; Associate Professor Department of Oncology, Pediatric Oncology and Radiotherapy 

2 Akkuratova St., S.-Petersburg, 197341, Russia; 2 Litovskaya St., S.-Petersburg, 194100, Russia

dinikina_yuv@almazovcentre.ruhttps://orcid.org/0000-0001-6667-3716КимА. В.KimA. V.

к.м.н., заведующий отделением нейрохирургии для детей № 7 НМИЦ им. В.А. Алмазова

197341, Санкт-Петербург, ул. Аккуратова, 2

Cand. of Sci. (Med.), Head of the Department of Pediatric Neurosurgery No. 7

2 Akkuratova St., S.-Petersburg, 197341, Russia

kimoza@mail.ruhttps://orcid.org/0000-0002-1635-6621ХачатрянВ. А.KhachatryanW. A.

д.м.н., профессор, член РАМТН, заслуженный деятель науки РФ, главный научный сотрудник отделения нейрохирургии детского возраста НИО нейрохирургии НМИЦ им. В.А. Алмазова

197341, Санкт-Петербург, ул. Аккуратова, 2

Dr. of Sci. (Med.), Professor, Member of the Russian Academy of Medical and Technical Sciences, Honored Worker of Science of the Russian Federation, Chief Researcher of the Department of Pediatric Neurosurgery

2 Akkuratova St., S.-Petersburg, 197341, Russia

wakhns@gmail.comhttps://orcid.org/0000-0002-7471-7181БелогуроваМ. Б.BelogurovaM. B.

Маргарита Борисовна Белогурова, д.м.н., профессор, ведущий научный сотрудник НИИ онкологии и гематологии НМИЦ им. В.А. Алмазова, заведующая кафедрой онкологии, детской онкологии и лучевой терапии СПбГПМУ

197341, Санкт-Петербург, ул. Аккуратова, 2; 194100, Санкт-Петербург, ул. Литовская, 2

Dr. of Sci. (Med.), Professor, Head of the Department of Oncology, Pediatric Oncology and Radiotherapy at the St. Petersburg State Pediatric Medical University, Leading Scientific Collaborator of Research Institute of Oncology and Hematology at the Almazov National Medical Research Center

2 Akkuratova St., S.-Petersburg, 197341, Russia; 2 Litovskaya St., S.-Petersburg, 194100, Russia

 

deton.hospital31@inbox.ruФГБУ «Национальный медицинский исследовательский центр имени В.А. Алмазова» Минздрава РоссииРоссияAlmazov National Medical Research Centre, Ministry of Health of RussiaRussian FederationФГБУ «Национальный медицинский исследовательский центр имени В.А. Алмазова» Минздрава России; ФГБОУ ВО «Санкт-Петербургский государственный педиатрический медицинский университет» Минздрава РоссииРоссияAlmazov National Medical Research Centre, Ministry of Health of Russia; Saint-Petersburg State Pediatric Medical University, Ministry of Health of RussiaRussian Federation202002032020714150Copyright © Смирнова А.Ю., Гончаров А.Ю., Диникина Ю.В., Ким А.В., Хачатрян В.А., Белогурова М.Б., 20202020Смирнова А.Ю., Гончаров А.Ю., Диникина Ю.В., Ким А.В., Хачатрян В.А., Белогурова М.Б.Smirnova A.Y., Goncharov A.Y., Dinikina Y.V., Kim A.V., Khachatryan W.A., Belogurova M.B.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/573

Атипические тератоидно-рабдоидные опухоли (АТРО) – злокачественные эмбриональные опухоли центральной нервной системы (ЦНС), встречающиеся преимущественно у детей раннего возраста. В 20–30 % случаев имеют место первично метастатические опухоли. ATРО является моногенным заболеванием, характеризующимся биаллельной мутацией гена SMARCB1, реже SMARCА4. Выживаемость пациентов с АТРО остается низкой. Применение мультимодальных методов лечения с включением хирургического этапа, стандартных режимов химиотерапии с интратекальным введением цитостатиков, высокодозной химиотерапии с поддержкой аутологичными стволовыми клетками и лучевой терапии имеет значительный потенциал для улучшения результатов. Недавние эпигенетические и транскрипционные исследования, проведенные двумя независимыми исследовательскими группами, позволяют выделить 3 подгруппы ATРО, имеющие различные молекулярные характеристики с соответствующей терапевтической чувствительностью. Дальнейшее изучение молекулярных вариантов, включение биологически направленных препаратов может являться многообещающей стратегией для оптимизации лечения в будущих исследованиях.

Atypical teratoid rhabdoid tumors (ATRT) are the most common malignant embryonal tumors of central nervous system in young children. Metastatic stage reveals in 20–30 % cases at the diagnosis. ATRT is a primarily monogenic disease characterized by the biallelic mutation of the SMARCB1, more rarely SMARCA4 genes. The survival rate of ATRT’s patients is poor. Multimodal treatment approaches including surgery, conventional dose chemotherapy with intrathecal therapy, high-dose chemotherapy with autologous stem cell resсue and radiotherapy have shown significant potential for improving outcomes. Recent epigenetic and transcriptional studies conducted by two independent research groups have identified three subgroups of ATRT. It has different molecular characteristics with appropriate therapeutic sensitivity. Further study of molecular types, inclusion of biologically targeted agents may be a promising strategy for optimizing treatment in future studies.

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The authors declare that there are no conflicts of interest present.