nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.17650/2311-1267-2018-5-4-60-73nodgo-442Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSАтипические тератоидно-рабдоидные опухоли центральной нервной системы у детей: состояние проблемы на сегодняшний день. Обзор литературыAtypical teratoid/rhabdoid tumors of the central nervous system in children: the state of the problem today. Literature reviewhttps://orcid.org/0000-0002-2003-0982ДиникинаЮ. В.DinikinaYu. V.

Юлия Валерьевна Диникина 

к.м.н., заведующая отделением химиотерапии онкогематологических заболеваний и трансплантации костного мозга для детей

доцент кафедры онкологии, детской онкологии, лучевой терапии 

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, Radiation Therapy 

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

д.м.н., профессор, ведущий научный сотрудник института гематологии

заведующая кафедрой онкологии, детской онкологии, лучевой терапии

M.B. Belogurova: Dr. of Sci. (Med.), Professor, Leading Scientific Collaborator of Institution of Hematology 

Head of the Department of Oncology, Pediatric Oncology, Radiotherapy 

deton.hospital31@inbox.ruФГБУ «Национальный медицинский исследовательский центр им. В.А. Алмазова» Минздрава РоссииРоссияAlmazov National Medical Research Centre, Ministry of Health of RussiaRussian FederationФГБОУ ВО «Санкт-Петербургский государственный педиатрический медицинский университет» Минздрава РоссииРоссияSaint-Petersburg State Pediatric Medical University, Ministry of Health of RussiaRussian Federation201822012019546073Copyright © Диникина Ю.В., Белогурова М.Б., 20192019Диникина Ю.В., Белогурова М.Б.Dinikina Y.V., 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/442

Атипические тератоидно-рабдоидные опухоли (АТРО) относятся к группе редких высоко агрессивных злокачественных опухолей у пациентов раннего возраста. Среди всех злокачественных опухолей центральной нервной системы (ЦНС) у детей они составляют 1–2 %, что ввиду малочисленности групп затрудняет разработку единых рекомендаций к противоопухолевой терапии. Достаточно изучен молекулярно-генетический профиль АТРО, во многом определяющий особенности заболевания. Несмотря на большое количество проводимых клинических исследований, результаты лечения АТРО ЦНС в мире на сегодняшний день остаются неудовлетворительными. Ранний возраст пациентов ограничивает использование лучевой терапии, что ведет к необходимости интенсификации режимов химиотерапии и выбора оптимальной стратегии в соотношении токсичность–польза. В статье описаны современные подходы к лечению АТРО ЦНС у детей, приведены результаты исследований с наибольшим числом включенных пациентов, с использованием мультимодальной стратегии терапии, обозначены современные направления таргетной терапии.

Конфликт интересов

Конфликт интересов. Авторы заявляют об отсутствии конфликта интересов. 

Финансирование

Финансирование. Исследование проведено без спонсорской поддержки. 

Atypical teratoid/rhabdoid tumors (AT/RT) are a group of rare highly aggressive malignant tumors in young patients. Among all the malignant tumors of the central nervous system (CNS) in children, they are 1–2 %, which, due to the small number of groups, makes it difficult to develop uniform recommendations for antitumor therapy. The molecular genetic profile of AT/RT, which largely determines the characteristics of the disease, has been studied sufficiently. Despite the large number of ongoing clinical studies, the results of treatment of AT/RT CNS in the world today remain unsatisfactory. The early age of patients limits the use of radiation therapy, which leads to the need to intensify chemotherapy regimens and to choose the optimal strategy in the toxicity – benefit ratio. The article describes modern approaches to the treatment of central nervous system disorders in children, presents the results of studies with the largest number of included patients, using the multimodal treatment strategy, identifies current trends in targeted therapy.

Conflict of interest

Conflict of interest. The authors declare no conflict of interest.

Funding

Funding. The study was performed without external funding.

атипичная тератоидно-рабдоидная опухольдетивысокодозная химиотерапиялучевая терапияопухоли центральной нервной системыatypical teratoid/rhabdoid tumorchildrenhigh-dose chemotherapyradiation therapytumors of the central nervous systemReferencesTekautz T., Fuller C., Blaney S., Foulad M., Fouladi M., Broniscer A., Merchant T.E., Krasin M., Dalton J., Hale G., Kun L.E., Wallace D., Gilbertson R.J., Gajjar A. Atypical Teratoid/Rhabdoid Tumors (ATRT): Improved Survival in Children 3 Years of Age and Older With Radiation Therapy and Hih-Dose Alkylator-Based Chemotherapy. J Clin Oncol 2005;23(7):1491–9. doi: 10.1200/JCO.2005.05.187.Tekautz T., Fuller C., Blaney S., Foulad M., Fouladi M., Broniscer A., Merchant T.E., Krasin M., Dalton J., Hale G., Kun L.E., Wallace D., Gilbertson R.J., Gajjar A. Atypical Teratoid/Rhabdoid Tumors (ATRT): Improved Survival in Children 3 Years of Age and Older With Radiation Therapy and Hih-Dose Alkylator-Based Chemotherapy. J Clin Oncol 2005;23(7):1491–9. doi: 10.1200/JCO.2005.05.187.Burger P.C., Yu I.T., Tihan T., Friedman H.S., Strother D.R., Kepner J.L., Duff ner P.K., Kun L.E., Perlman E.J. Atypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group study. Am J Surg Pathol 1998;22(9):1083–92. PMID: 9737241.Burger P.C., Yu I.T., Tihan T., Friedman H.S., Strother D.R., Kepner J.L., Duff ner P.K., Kun L.E., Perlman E.J. Atypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group study. Am J Surg Pathol 1998;22(9):1083–92. PMID: 9737241.Von Hoff K., Hinkes B., Dannenmann-Stern E., von Bueren A.O., Warmuth-Metz M., Soerensen N., Emser A., Zwiener I., Schlegel P.G., Kuehl J., Frühwald M.C., Kortmann R.D., Pietsch T., Rutkowski S. Frequency, risk-factors and survival of children with atypical teratoid rhabdoid tumors (AT/RT) of the CNS diagnosed between 1988 and 2004, and registered to the German HIT database. Pediatr Blood Cancer 2011;57(6):978–85. doi: 10.1002/pbc.23236.Von Hoff K., Hinkes B., Dannenmann-Stern E., von Bueren A.O., Warmuth-Metz M., Soerensen N., Emser A., Zwiener I., Schlegel P.G., Kuehl J., Frühwald M.C., Kortmann R.D., Pietsch T., Rutkowski S. Frequency, risk-factors and survival of children with atypical teratoid rhabdoid tumors (AT/RT) of the CNS diagnosed between 1988 and 2004, and registered to the German HIT database. Pediatr Blood Cancer 2011;57(6):978–85. doi: 10.1002/pbc.23236.Hilden J., Meerbaum S., Burger P., Finlay J., Janss A., Scheithauer B.W., Walter A.W., Rorke L.B., Biegel J.A. Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry. J Clin Oncol 2004;22(14):2877–84. doi: 10.1200/JCO.2004.07.073.Hilden J., Meerbaum S., Burger P., Finlay J., Janss A., Scheithauer B.W., Walter A.W., Rorke L.B., Biegel J.A. Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry. J Clin Oncol 2004;22(14):2877–84. doi: 10.1200/JCO.2004.07.073.Lafay-Cousin L., Hawkins C., Carret A., Johnston D., Zelcer S., Wilson B., Jabado N., Scheinemann K., Eisenstat D., Fryer C., Fleming A., Mpofu C., Larouche V., Strother D., Bouff et E., Huang A. Central nervous system atypical teratoid rhabdoid tumours: the Canadian Paediatric Brain Tumour Consortium experience. Eur J Cancer 2012;48(3):353–9. doi: 10.1016/j.ejca.2011.09.005.Lafay-Cousin L., Hawkins C., Carret A., Johnston D., Zelcer S., Wilson B., Jabado N., Scheinemann K., Eisenstat D., Fryer C., Fleming A., Mpofu C., Larouche V., Strother D., Bouff et E., Huang A. Central nervous system atypical teratoid rhabdoid tumours: the Canadian Paediatric Brain Tumour Consortium experience. Eur J Cancer 2012;48(3):353–9. doi: 10.1016/j.ejca.2011.09.005.Михайленко Д.С., Телешова М.В., Перепечин Д.В., Ефремов Г.Д., Качанов Д.Ю., Райкина Е.В., Бобрынина В.О., Лаврина С.Г., Митрофанова А.М., Коновалов Д.М., Варфоломеева С.Р., Алексеев Б.Я. Герминальные нонсенс-мутации в гене SMARCB1 у российских пациентов с рабдоидными опухолями почек. Онкоурология 2017;3:14–9. doi: 10.17650/1726-9776-2017-13-2-14-19. [Mikhaylenko D.S., Teleshova M.V., Perepechin D.V., Efremov G.D., Kachanov D.Yu., Raykina E.V., Bobrynina V.O., Lavrina S.G., Mitrofanova A.M., Konovalov D.M., Varfolomeeva S.R., Alekseev B.Ya. Germline nonsensemutations of the SMARCB1 gene in Russian patients with rhabdoid renal tumors. Onkourologiya = Oncourology 2017;3:14–9. (In Russ.)].Михайленко Д.С., Телешова М.В., Перепечин Д.В., Ефремов Г.Д., Качанов Д.Ю., Райкина Е.В., Бобрынина В.О., Лаврина С.Г., Митрофанова А.М., Коновалов Д.М., Варфоломеева С.Р., Алексеев Б.Я. Герминальные нонсенс-мутации в гене SMARCB1 у российских пациентов с рабдоидными опухолями почек. Онкоурология 2017;3:14–9. doi: 10.17650/1726-9776-2017-13-2-14-19. [Mikhaylenko D.S., Teleshova M.V., Perepechin D.V., Efremov G.D., Kachanov D.Yu., Raykina E.V., Bobrynina V.O., Lavrina S.G., Mitrofanova A.M., Konovalov D.M., Varfolomeeva S.R., Alekseev B.Ya. Germline nonsensemutations of the SMARCB1 gene in Russian patients with rhabdoid renal tumors. Onkourologiya = Oncourology 2017;3:14–9. (In Russ.)].Bourdeaut F., Lequin D., Brugieres L., Reynaud S., Dufour C., Doz F., André N., Stephan J.L., Pérel Y., Oberlin O., Orbach D., Bergeron C., Rialland X., Fréneaux P., Ranchere D., Figarella-Branger D., Audry G., Puget S., Evans D.G., Pinas J.C., Capra V., Mosseri V., Coupier I., Gauthier-Villars M., Pierron G., Delattre O. Frequent hSNF5/INI1 germline mutations in patients with rhabdoid tumor. Clin Cancer Res 2011;17(1):31–8. doi: 10.1158/1078-0432.CCR-10-1795.Bourdeaut F., Lequin D., Brugieres L., Reynaud S., Dufour C., Doz F., André N., Stephan J.L., Pérel Y., Oberlin O., Orbach D., Bergeron C., Rialland X., Fréneaux P., Ranchere D., Figarella-Branger D., Audry G., Puget S., Evans D.G., Pinas J.C., Capra V., Mosseri V., Coupier I., Gauthier-Villars M., Pierron G., Delattre O. Frequent hSNF5/INI1 germline mutations in patients with rhabdoid tumor. Clin Cancer Res 2011;17(1):31–8. doi: 10.1158/1078-0432.CCR-10-1795.Митрофанова А.М., Коновалов Д.М., Кисляков А.Н., Рощин В.Ю., Абрамов Д.С., Рогожин Д.В. Атипичные тератоидно-рабдоидные опухоли детского возраста. Архив патологии 2013;5:36–42. [Mitrofanova A.M., Konovalov D.M., Kislyakov A.N., Roshchin V.Yu., Abramov D.S., Rogozhin D.V. Atypical teratoid/rhabdoid tumors of childhood. Arkhiv patologii = Pathology Archive 2013;5:36–42. (In Russ.)].Митрофанова А.М., Коновалов Д.М., Кисляков А.Н., Рощин В.Ю., Абрамов Д.С., Рогожин Д.В. Атипичные тератоидно-рабдоидные опухоли детского возраста. Архив патологии 2013;5:36–42. [Mitrofanova A.M., Konovalov D.M., Kislyakov A.N., Roshchin V.Yu., Abramov D.S., Rogozhin D.V. Atypical teratoid/rhabdoid tumors of childhood. Arkhiv patologii = Pathology Archive 2013;5:36–42. (In Russ.)].Пьянзин С.Ю., Сивцова Е.В. Атипическая тератоидно-рабдоидная опухоль головного мозга у детей. Нейрохирургия 2007;4:47–50. [Pyanzin S.Yu., Sivtsova E.V. Atypical teratoid-rhabdoid brain tumor in children. Neyrokhirurgiya = Neurosurgery 2007;4:47–50. (In Russ.)].Пьянзин С.Ю., Сивцова Е.В. Атипическая тератоидно-рабдоидная опухоль головного мозга у детей. Нейрохирургия 2007;4:47–50. [Pyanzin S.Yu., Sivtsova E.V. Atypical teratoid-rhabdoid brain tumor in children. Neyrokhirurgiya = Neurosurgery 2007;4:47–50. (In Russ.)].Frühwald M.C., Biegel J.A., Bourdeaut F., Roberts C., Chi S.N. Atypical teratoid/rhabdoid tumors – current concepts, advances in biology, and potential future therapies. Neuro Oncol 2016;18(6):764–78. doi: 10.1093/neuonc/nov264.Frühwald M.C., Biegel J.A., Bourdeaut F., Roberts C., Chi S.N. Atypical teratoid/rhabdoid tumors – current concepts, advances in biology, and potential future therapies. Neuro Oncol 2016;18(6):764–78. doi: 10.1093/neuonc/nov264.Hasselblatt M., Isken S., Linge A., Eikmeier K., Jeibmann A., Oyen F., Nagel I., Richter J., Bartelheim K., Kordes U., Schneppenheim R., Frühwald M., Siebert R., Paulus W. High-resolution genomic analysis suggests the absence of recurrent genomic alterations other than SMARCB1 aberrations in atypical teratoid/rhabdoid tumors. Genes Chromosomes Cancer 2013;52(2):185–90. doi: 10.1002/gcc.22018.Hasselblatt M., Isken S., Linge A., Eikmeier K., Jeibmann A., Oyen F., Nagel I., Richter J., Bartelheim K., Kordes U., Schneppenheim R., Frühwald M., Siebert R., Paulus W. High-resolution genomic analysis suggests the absence of recurrent genomic alterations other than SMARCB1 aberrations in atypical teratoid/rhabdoid tumors. Genes Chromosomes Cancer 2013;52(2):185–90. doi: 10.1002/gcc.22018.Ginn K., Gaajjar A. Atypical teratoid rhabdoid tumor: current therapy and future directions. Front Oncol 2012;2:114. doi: 10.3389/fonc.2012.00114.Ginn K., Gaajjar A. Atypical teratoid rhabdoid tumor: current therapy and future directions. Front Oncol 2012;2:114. doi: 10.3389/fonc.2012.00114.Kohashi K., Oda Y. Oncogenic roles of SMARCB1/INI1 and its defi cient tumors. Cancer Sci 2017;108(4):547–52. doi: 10.1111/cas.13173.Kohashi K., Oda Y. Oncogenic roles of SMARCB1/INI1 and its defi cient tumors. Cancer Sci 2017;108(4):547–52. doi: 10.1111/cas.13173.Chi S., Zimmerman M., Yao X., Cohen K.J., Burger P., Biegel J.A., Rorke-Adams L.B., Fisher M.J., Janss A., Mazewski C., Goldman S., Manley P.E., Bowers D.C., Bendel A., Rubin J., Turner C.D., Marcus K.J., Goumnerova L., Ullrich N.J., Kieran M.W. Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol 2009;27(3):385–9. doi: 10.1200/JCO.2008.18.7724.Chi S., Zimmerman M., Yao X., Cohen K.J., Burger P., Biegel J.A., Rorke-Adams L.B., Fisher M.J., Janss A., Mazewski C., Goldman S., Manley P.E., Bowers D.C., Bendel A., Rubin J., Turner C.D., Marcus K.J., Goumnerova L., Ullrich N.J., Kieran M.W. Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol 2009;27(3):385–9. doi: 10.1200/JCO.2008.18.7724.Dufour C., Beaugrand A., Le Deley M., Bourdeaut .F, André N., Leblond P., Bertozzi A.I., Frappaz D., Rialland X., Fouyssac F., Edan C., Grill J., Quidot M., Varlet P. Clinicopathologic prognostic factors in childhood atypical teratoid and rhabdoid tumor of the central nervous system. Cancer 2011;118(15):3812–21. doi: 10.1002/cncr.26684.Dufour C., Beaugrand A., Le Deley M., Bourdeaut .F, André N., Leblond P., Bertozzi A.I., Frappaz D., Rialland X., Fouyssac F., Edan C., Grill J., Quidot M., Varlet P. Clinicopathologic prognostic factors in childhood atypical teratoid and rhabdoid tumor of the central nervous system. Cancer 2011;118(15):3812–21. doi: 10.1002/cncr.26684.Slavc I., Chocholous M., Leiss U., Haberler C., Peyrl A., Azizi A., Dieckmann K., Woehrer A., Peters C., Widhalm G., Dorfer Ch., Czech T. Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992–2012. Cancer Med 2014;3(1):91–100. doi: 10.1002/cam4.161.Slavc I., Chocholous M., Leiss U., Haberler C., Peyrl A., Azizi A., Dieckmann K., Woehrer A., Peters C., Widhalm G., Dorfer Ch., Czech T. Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992–2012. Cancer Med 2014;3(1):91–100. doi: 10.1002/cam4.161.Bartelheim K., Nemes K., Seeringer A., Kerl K., Buechner J., Boos J., Graf N., Dürken M., Gerss J., Hasselblatt M., Kortmann R., Teichert von Luettichau I., Nagel I., Nygaard R., Oyen F., Quiroga E., Schlegel PG., Schmid I., Schneppenheim R., Siebert R., Solano-Paez P., Timmermann B., Warmuth-Metz M., Frühwald M. Improved 6-year overall survival in AT/RT-results of the registry study Rhabdoid 2007. Cancer Med 2016;5(8):1765–75. doi: 10.1002/cam4.741.Bartelheim K., Nemes K., Seeringer A., Kerl K., Buechner J., Boos J., Graf N., Dürken M., Gerss J., Hasselblatt M., Kortmann R., Teichert von Luettichau I., Nagel I., Nygaard R., Oyen F., Quiroga E., Schlegel PG., Schmid I., Schneppenheim R., Siebert R., Solano-Paez P., Timmermann B., Warmuth-Metz M., Frühwald M. Improved 6-year overall survival in AT/RT-results of the registry study Rhabdoid 2007. Cancer Med 2016;5(8):1765–75. doi: 10.1002/cam4.741.Ostrom Q.T., de Blank P.M., Kruchko C., Petersen C.M., Liao P., Finlay J.L., Stearns D.S., Wolff J.E., Wolinsky Y., Letterio J.J., Barnholtz-Sloan J.S. Alex’s Lemonade Stand Foundation infant and Childhodd primary brain and central nervous system tumors diagnosed in the United States in 2007–2011. Neuro Oncol 2005;23(7):1491–9. doi: 10.1093/neuonc/nou327.Ostrom Q.T., de Blank P.M., Kruchko C., Petersen C.M., Liao P., Finlay J.L., Stearns D.S., Wolff J.E., Wolinsky Y., Letterio J.J., Barnholtz-Sloan J.S. Alex’s Lemonade Stand Foundation infant and Childhodd primary brain and central nervous system tumors diagnosed in the United States in 2007–2011. Neuro Oncol 2005;23(7):1491–9. doi: 10.1093/neuonc/nou327.Judkins A.R., Eberhart C.G., Wesseling P. Atypical teratoid/rhabdoid tumor. In: World Health Organization classifi cation of tumors: pathology ang genetics of central nervous system. Lyon: IARC Press, 2007. Pp. 147–149.Judkins A.R., Eberhart C.G., Wesseling P. Atypical teratoid/rhabdoid tumor. In: World Health Organization classifi cation of tumors: pathology ang genetics of central nervous system. Lyon: IARC Press, 2007. Pp. 147–149.Buscariollo D.L., Pak H.S., Roberts K.B., Yu J.B. Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance, Epidemiology, and End Results anlysis. Cancer 2012;118(17):4212–9. doi: 10.1002/cncr.27373.Buscariollo D.L., Pak H.S., Roberts K.B., Yu J.B. Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance, Epidemiology, and End Results anlysis. Cancer 2012;118(17):4212–9. doi: 10.1002/cncr.27373.Chen M.L., McComb J.G., Krieger M.D. Atypical teratoid/rhabdoid tumors of the central nervous system: management and outcomes. Neurosurg Focus 2005;18(6A):E8. PMID: 16048294.Chen M.L., McComb J.G., Krieger M.D. Atypical teratoid/rhabdoid tumors of the central nervous system: management and outcomes. Neurosurg Focus 2005;18(6A):E8. PMID: 16048294.Warmuth-Metz M., Bison B., Dannemann-Stern E., Kortmann R., Rutkowski S., Pietsch T. CT and MRI imaging in atypical teratoid/ rhabdoid tumors of the central nervous system. Neuroradiology 2008;50(5):447–52. doi: 10.1007/s00234-008-0369-7.Warmuth-Metz M., Bison B., Dannemann-Stern E., Kortmann R., Rutkowski S., Pietsch T. CT and MRI imaging in atypical teratoid/ rhabdoid tumors of the central nervous system. Neuroradiology 2008;50(5):447–52. doi: 10.1007/s00234-008-0369-7.Meyers S., Khademian Z., Biegel J., Chuang S.H., Korones D.N., Zimmerman R.A. Primary Intracranial Atypical Teratoid/Rhabdoid Tumors of Infancy and Childhood: MRI Features and Patient Outcomes. AJNR Am J Neuroradiol 2006;27(5):962–71. PMID: 16687525.Meyers S., Khademian Z., Biegel J., Chuang S.H., Korones D.N., Zimmerman R.A. Primary Intracranial Atypical Teratoid/Rhabdoid Tumors of Infancy and Childhood: MRI Features and Patient Outcomes. AJNR Am J Neuroradiol 2006;27(5):962–71. PMID: 16687525.Biswas A., Julka K., Bakhshi S., Suri A., Rath G.K. Intracranial atypical teratoid rhabdoid tumor: current management and a single institute experience of 15 patients from north India. Acta Neurochirurgica 2015;157(4):589–96. doi: 10.1007/s00701-015-2355-2.Biswas A., Julka K., Bakhshi S., Suri A., Rath G.K. Intracranial atypical teratoid rhabdoid tumor: current management and a single institute experience of 15 patients from north India. Acta Neurochirurgica 2015;157(4):589–96. doi: 10.1007/s00701-015-2355-2.Cheng Y., Lirng J., Chang F., Guo W.Y., Teng M.M., Chang C.Y., Wong T.T., Ho D.M. Neuroradiological fi ndings in atypical teratoid/rhabdoid tumor of the central nervous system. Acta Radiol 2005;46(1):89–96. PMID: 15841745.Cheng Y., Lirng J., Chang F., Guo W.Y., Teng M.M., Chang C.Y., Wong T.T., Ho D.M. Neuroradiological fi ndings in atypical teratoid/rhabdoid tumor of the central nervous system. Acta Radiol 2005;46(1):89–96. PMID: 15841745.Meyers S., Wildenhain S., Chang J., Bourekas E.C., Beattie P.F., Korones D.N., Davis D., Pollack I.F., Zimmerman R.A. Postoperative evaluation for disseminated medulloblastoma involving the spine: contrast-enhanced MR fi ndings, CSF cytologic analysis, timing of disease occurrence, and patient outcomes. AJNR Am J Neuroradiol 2000;21:1757–65. PMID: 11039362.Meyers S., Wildenhain S., Chang J., Bourekas E.C., Beattie P.F., Korones D.N., Davis D., Pollack I.F., Zimmerman R.A. Postoperative evaluation for disseminated medulloblastoma involving the spine: contrast-enhanced MR fi ndings, CSF cytologic analysis, timing of disease occurrence, and patient outcomes. AJNR Am J Neuroradiol 2000;21:1757–65. PMID: 11039362.Gajjar A., Fouladi M., Walter A., Thompson S.J., Reardon D.A., Merchant T.E., Jenkins J.J., Liu A., Boyett J.M., Kun L.E., Heideman R.L. Comparison of lumbar and shunt cerebrospinal fl uid specimens for cytologic detection of leptomeningeal disease in pediatric patients with brain tumors. J Clin Oncol 1999;17:1825–8. doi: 10.1200/JCO.1999.17.6.1825.Gajjar A., Fouladi M., Walter A., Thompson S.J., Reardon D.A., Merchant T.E., Jenkins J.J., Liu A., Boyett J.M., Kun L.E., Heideman R.L. Comparison of lumbar and shunt cerebrospinal fl uid specimens for cytologic detection of leptomeningeal disease in pediatric patients with brain tumors. J Clin Oncol 1999;17:1825–8. doi: 10.1200/JCO.1999.17.6.1825.Shalaby T., Achini F., Grotzer M. Targeting cerebrospinal fl uid for discovery of brain cancer biomarkers. J Cancer Metastasis Treat 2016;2:176–87. doi: 10.20517/2394-4722.2016.12.Shalaby T., Achini F., Grotzer M. Targeting cerebrospinal fl uid for discovery of brain cancer biomarkers. J Cancer Metastasis Treat 2016;2:176–87. doi: 10.20517/2394-4722.2016.12.Terterov S., Krieger M.D., Bowen I., McComb J.G. Evaluation of intracranial cerebrospinal fl uid cytology in staging pediatric medulloblastomas, supratentorial primitive neuroectodermal tumors, and ependymomas. J Neurosurg Pediatr 2010;6:131–6. doi: 10.3171/2010.5. PEDS 09333.Terterov S., Krieger M.D., Bowen I., McComb J.G. Evaluation of intracranial cerebrospinal fl uid cytology in staging pediatric medulloblastomas, supratentorial primitive neuroectodermal tumors, and ependymomas. J Neurosurg Pediatr 2010;6:131–6. doi: 10.3171/2010.5. PEDS 09333.Sredni S., Tomita T. Rhabdoid tumor predisposition syndrome. Pediatr Dev Pathol 2015;18(1):49–58. doi: 10.2350/14-07-1531-MISC.1.Sredni S., Tomita T. Rhabdoid tumor predisposition syndrome. Pediatr Dev Pathol 2015;18(1):49–58. doi: 10.2350/14-07-1531-MISC.1.Михайленко Д.С., Телешова М.В., Ефремов Г.Д., Алексеев Б.Я. Мутации гена SMARCB1 в опухолях различной локализации. Альманах клинической медицины 2016;44(5):558–67. doi: 10.18786/2072-0505- 2016-44-5-558-567. [Mikhaylenko D.S., Teleshova M.V., Efremov G.D., Alekseev B.Ya. Mutations of the SMARCB1 gene in human cancers. Al’manakh klinicheskoy meditsiny = Almanac of Clinical Medicine 2016;44(5):558–67. (In Russ.)].Михайленко Д.С., Телешова М.В., Ефремов Г.Д., Алексеев Б.Я. Мутации гена SMARCB1 в опухолях различной локализации. Альманах клинической медицины 2016;44(5):558–67. doi: 10.18786/2072-0505- 2016-44-5-558-567. [Mikhaylenko D.S., Teleshova M.V., Efremov G.D., Alekseev B.Ya. Mutations of the SMARCB1 gene in human cancers. Al’manakh klinicheskoy meditsiny = Almanac of Clinical Medicine 2016;44(5):558–67. (In Russ.)].Witkowski L., Lalonde E., Zhang J., Albrecht S., Hamel N., Cavallone L., May S.T., Nicholson J.C., Coleman N., Murray M.J., Tauber P.F., Huntsman D.G., Schönberger S., Yandell D., Hasselblatt M., Tischkowitz M.D., Majewski J., Foulkes W.D. Familial rhabdoid tumor ‘avant la lettre’– from pathology review to exome sequencing and back again. J Pathol 2013;231(1):35–43. doi: 10.1002/path.4225.Witkowski L., Lalonde E., Zhang J., Albrecht S., Hamel N., Cavallone L., May S.T., Nicholson J.C., Coleman N., Murray M.J., Tauber P.F., Huntsman D.G., Schönberger S., Yandell D., Hasselblatt M., Tischkowitz M.D., Majewski J., Foulkes W.D. Familial rhabdoid tumor ‘avant la lettre’– from pathology review to exome sequencing and back again. J Pathol 2013;231(1):35–43. doi: 10.1002/path.4225.Johansson G., Andersson U., Melin B. Recent developments in brain tumor predisposing syndromes. Acta Oncol 2016;55(4):401–11. doi: 10.3109/0284186X.2015.1107190.Johansson G., Andersson U., Melin B. Recent developments in brain tumor predisposing syndromes. Acta Oncol 2016;55(4):401–11. doi: 10.3109/0284186X.2015.1107190.Kordes U., Gesk S., Fruhwald M., Graf N., Leuschner I., Hasselblatt M., Jeibmann A., Oyen F., Peters O., Pietsch T., Siebert R., Schneppenheim R. Clinical and molecular features in patients with atypical teratoid rhabdoid tumor or malignant rhabdoid tumor. Genes Chromosomes Cancer 2010;49(2):176–81. doi: 10.1002/gcc.20729.Kordes U., Gesk S., Fruhwald M., Graf N., Leuschner I., Hasselblatt M., Jeibmann A., Oyen F., Peters O., Pietsch T., Siebert R., Schneppenheim R. Clinical and molecular features in patients with atypical teratoid rhabdoid tumor or malignant rhabdoid tumor. Genes Chromosomes Cancer 2010;49(2):176–81. doi: 10.1002/gcc.20729.Biegel J., Busse T., Weissman B. SWI/SNF chromatin remodeling complexes and cancer. Am J Med Genet C Semin Med Genet 2014;166C(3):350–366. doi: 10.1002/ajmg.c.31410.Biegel J., Busse T., Weissman B. SWI/SNF chromatin remodeling complexes and cancer. Am J Med Genet C Semin Med Genet 2014;166C(3):350–366. doi: 10.1002/ajmg.c.31410.Ammerlaan A., Arariu A., Hoben M., Baas F., Tijssen C., Teepen J., Wesseling P., Hulsebos T. Long-term survival and transmission of INI1- mutation via nonpenetrant males in a family with rhabdoid tumor predisposition syndrome. Br J Cancer 2008;98(2):474–9. doi: 10.1038/sj.bjc.6604156.Ammerlaan A., Arariu A., Hoben M., Baas F., Tijssen C., Teepen J., Wesseling P., Hulsebos T. Long-term survival and transmission of INI1- mutation via nonpenetrant males in a family with rhabdoid tumor predisposition syndrome. Br J Cancer 2008;98(2):474–9. doi: 10.1038/sj.bjc.6604156.Frühwald M., Hasselblatt M., Wirth S., Köhler G., Schneppenheim R., Subero J.I., Siebert R., Kordes U., Jürgens H., Vormoor J. Non-linkage of familial rhabdoid tumors to SMARCB1 implies a second locus for the rhabdoid tumor predisposition syndrome. Pediatr Blood Cancer 2006;47(3):273–8. doi: 10.1002/pbc.20526.Frühwald M., Hasselblatt M., Wirth S., Köhler G., Schneppenheim R., Subero J.I., Siebert R., Kordes U., Jürgens H., Vormoor J. Non-linkage of familial rhabdoid tumors to SMARCB1 implies a second locus for the rhabdoid tumor predisposition syndrome. Pediatr Blood Cancer 2006;47(3):273–8. doi: 10.1002/pbc.20526.Kordes U., Bartelheim K., Modena P., Massimino M., Biassoni V., Reinhard H., Hasselblatt M., Schneppenheim R., Frühwald M.C. Favorable outcome of patients aff ected by rhabdoid tumors due to rhabdoid tumor predisposition syndrome (RTPS). Pediatr Blood Cancer 2014;61(5):919–21. doi: 10.1002/pbc.24793.Kordes U., Bartelheim K., Modena P., Massimino M., Biassoni V., Reinhard H., Hasselblatt M., Schneppenheim R., Frühwald M.C. Favorable outcome of patients aff ected by rhabdoid tumors due to rhabdoid tumor predisposition syndrome (RTPS). Pediatr Blood Cancer 2014;61(5):919–21. doi: 10.1002/pbc.24793.Torchia J., Picard D., Lafay-Cousin L., Hawkins C.E., Kim S.K., Letourneau L., Ra Y.S., Ho K.C., Chan T.S., Sin-Chan P., Dunham C.P., Yip S., Ng H.K., Lu J.Q., Albrecht S., Pimentel J., Chan J.A., Somers G., Zielenska M., Faria C.C., Roque L., Baskin B., Birks D., Foreman N., Strother D., Klekner A., Garami M., Hauser P., Hortobágyi T., Bognár L., Wilson B., Hukin J., Carret A.S., Van Meter T.E., Nakamura H., Toledano H., Fried I., Fults D., Wataya T., Fryer C., Eisenstat D.D., Scheineman K., Johnston D., Michaud J., Zelcer S., Hammond R., Ramsay D.A., Fleming A.J., Lulla R.R., Fangusaro J.R., Sirachainan N., Larbcharoensub N., Hongeng S., Barakzai M.A., Montpetit A., Stephens D., Grundy R.G., Schüller U., Nicolaides T., Tihan T., Phillips J., Taylor M.D., Rutka J.T., Dirks P., Bader G.D., Warmuth-Metz M., Rutkowski S., Pietsch T., Judkins A.R., Jabado N., Bouff et E., Huang A. Molecular subgroups of atypical teratoid rhabdoid tumours in children: an intergrated genomic and clinicopathological analysis. Lancet Oncol 2015;16(5):569–82. doi: 10.1016/S1470-2045(15)70114-2.Torchia J., Picard D., Lafay-Cousin L., Hawkins C.E., Kim S.K., Letourneau L., Ra Y.S., Ho K.C., Chan T.S., Sin-Chan P., Dunham C.P., Yip S., Ng H.K., Lu J.Q., Albrecht S., Pimentel J., Chan J.A., Somers G., Zielenska M., Faria C.C., Roque L., Baskin B., Birks D., Foreman N., Strother D., Klekner A., Garami M., Hauser P., Hortobágyi T., Bognár L., Wilson B., Hukin J., Carret A.S., Van Meter T.E., Nakamura H., Toledano H., Fried I., Fults D., Wataya T., Fryer C., Eisenstat D.D., Scheineman K., Johnston D., Michaud J., Zelcer S., Hammond R., Ramsay D.A., Fleming A.J., Lulla R.R., Fangusaro J.R., Sirachainan N., Larbcharoensub N., Hongeng S., Barakzai M.A., Montpetit A., Stephens D., Grundy R.G., Schüller U., Nicolaides T., Tihan T., Phillips J., Taylor M.D., Rutka J.T., Dirks P., Bader G.D., Warmuth-Metz M., Rutkowski S., Pietsch T., Judkins A.R., Jabado N., Bouff et E., Huang A. Molecular subgroups of atypical teratoid rhabdoid tumours in children: an intergrated genomic and clinicopathological analysis. Lancet Oncol 2015;16(5):569–82. doi: 10.1016/S1470-2045(15)70114-2.Birks D., Donson A., Patel P., Dunham C., Muscat A., Algar E.M., Ashley D.M., Kleinschmidt-Demasters B.K., Vibhakar R., Handler M.H., Foreman N.K. High expression of BMP pathway genes distinguishes a subset of atypical teratoid/rhabdoid tumors associated with shorter survival. Neuro Oncol 2011;13(12):1296–307. doi: 10.1093/neuonc/nor140.Birks D., Donson A., Patel P., Dunham C., Muscat A., Algar E.M., Ashley D.M., Kleinschmidt-Demasters B.K., Vibhakar R., Handler M.H., Foreman N.K. High expression of BMP pathway genes distinguishes a subset of atypical teratoid/rhabdoid tumors associated with shorter survival. Neuro Oncol 2011;13(12):1296–307. doi: 10.1093/neuonc/nor140.Johann P., Erkek S., Zapatka M., Kerl K., Buchhalter I., Hovestadt V., Jones D.T.W., Sturm D., Hermann C., Segura Wang M., Korshunov A., Rhyzova M., Gröbner S., Brabetz S., Chavez L., Bens S., Gröschel S., Kratochwil F., Wittmann A., Sieber L., Geörg C., Wolf S., Beck K., Oyen F., Capper D., van Sluis P., Volckmann R., Koster J., Versteeg R., von Deimling A., Milde T., Witt O., Kulozik A.E., Ebinger M., Shalaby T., Grotzer M., Sumerauer D., Zamecnik J., Mora J., Jabado N., Taylor M.D., Huang A., Aronica E., Bertoni A., Radlwimmer B., Pietsch T., Schüller U., Schneppenheim R., Northcott P.A., Korbel J.O., Siebert R., Frühwald M.C., Lichter P., Eils R., Gajjar A., Hasselblatt M., Pfi ster S.M., Kool M. Atypical teratoid/rhabdoid tumors are comprised of three epigenetic subgroups with distinct enhancer landscapes. Cancer Cell 2016;29(3):379–93. doi: 10.1016/j.ccell.2016.02.001.Johann P., Erkek S., Zapatka M., Kerl K., Buchhalter I., Hovestadt V., Jones D.T.W., Sturm D., Hermann C., Segura Wang M., Korshunov A., Rhyzova M., Gröbner S., Brabetz S., Chavez L., Bens S., Gröschel S., Kratochwil F., Wittmann A., Sieber L., Geörg C., Wolf S., Beck K., Oyen F., Capper D., van Sluis P., Volckmann R., Koster J., Versteeg R., von Deimling A., Milde T., Witt O., Kulozik A.E., Ebinger M., Shalaby T., Grotzer M., Sumerauer D., Zamecnik J., Mora J., Jabado N., Taylor M.D., Huang A., Aronica E., Bertoni A., Radlwimmer B., Pietsch T., Schüller U., Schneppenheim R., Northcott P.A., Korbel J.O., Siebert R., Frühwald M.C., Lichter P., Eils R., Gajjar A., Hasselblatt M., Pfi ster S.M., Kool M. Atypical teratoid/rhabdoid tumors are comprised of three epigenetic subgroups with distinct enhancer landscapes. Cancer Cell 2016;29(3):379–93. doi: 10.1016/j.ccell.2016.02.001.Chen Y., Wong T., Ho D., Huang P.I., Chang K.P., Shiau C.Y., Yen S.H. Impact of radiotherapy for pediatric CNS atypical theratoid/rhabdoid tumor (single institute experience). Int J Radiat Oncol Biol Phys 2006;64(4):1038–43. doi: 10.1016/j.ijrobp.2005.10.001.Chen Y., Wong T., Ho D., Huang P.I., Chang K.P., Shiau C.Y., Yen S.H. Impact of radiotherapy for pediatric CNS atypical theratoid/rhabdoid tumor (single institute experience). Int J Radiat Oncol Biol Phys 2006;64(4):1038–43. doi: 10.1016/j.ijrobp.2005.10.001.Di Rocco C., Massimi L. Atypical Teratoid/Rhabdoid Tumor: Surgery. Division of Pediatric Neurosurgery Catholic University, Medical SchoolRome, Italy, 2015. Pp. 643–650. https://doi.org/10.1007/978-3-319-11274-9_40.Di Rocco C., Massimi L. Atypical Teratoid/Rhabdoid Tumor: Surgery. Division of Pediatric Neurosurgery Catholic University, Medical SchoolRome, Italy, 2015. Pp. 643–650. https://doi.org/10.1007/978-3-319-11274-9_40.Gupta N., Banerjee A., Haas-Kogan D. Pediatric CNS Tumors. 2nd edition. Springer-Verlag Berlin Heidelberg, 2010. Pp. 273–283.Gupta N., Banerjee A., Haas-Kogan D. Pediatric CNS Tumors. 2nd edition. Springer-Verlag Berlin Heidelberg, 2010. Pp. 273–283.Кумирова Э.В., Желудкова О.Г., Бородина И.Д., Горбатых С.В., Лившиц М.И., Попов В.Е., Коршунов А.Г., Меликян А.Г., Кушель Ю.В., Озерова В.И., Озеров С.С., Горелышев С.К., Щербенко О.И., Зелинская Н.И., Полушкина О.Б., Говорина Е.В., Холодов Б.В., Тарасова Е.М., Купцова Е.В., Привалова Л.П., Карелин А.Ф., Шаммасов Р.З., Гришина Е.Н., Стремнева О.В., Белогурова М.Б., Шац Л.И., Мушинская М.В., Павлова Е.В., Мазеркина Н.А., Румянцев А.Г. Результаты лечения редких эмбриональных опухолей ЦНС у детей. Педиатрия 2008;87(4):64–8. [Kumirova E.V., Zheludkova O.G., Borodina I.D., Gorbatykh S.V., Livshits M.I., Popov V.E., Korshunov A.G., Melikyan A.G., Kushel Yu .V., Ozerova V.I., Ozerov S.S., Gorelyshev S.K., Shcherbenko O.I., Zelinskaya N.I., Polushkina O.B., Govorina E.V., Kholodov B.V., Tarasova E.M., Kuptsova E.V., Privalova L.P., Karelin A.F., Shammasov R.Z., Grishina E.N., Stremneva O.V., Belogurova M.B., Schatz L.I., Mushinskaya M.V., Pavlova E.V., Mazerkina N.A., Rumyantsev A.G. Results of treatment of rare CNS embryonic tumors in children. Pediatriya = Pediatrics 2008;87(4):64–68. (In Russ.)].Кумирова Э.В., Желудкова О.Г., Бородина И.Д., Горбатых С.В., Лившиц М.И., Попов В.Е., Коршунов А.Г., Меликян А.Г., Кушель Ю.В., Озерова В.И., Озеров С.С., Горелышев С.К., Щербенко О.И., Зелинская Н.И., Полушкина О.Б., Говорина Е.В., Холодов Б.В., Тарасова Е.М., Купцова Е.В., Привалова Л.П., Карелин А.Ф., Шаммасов Р.З., Гришина Е.Н., Стремнева О.В., Белогурова М.Б., Шац Л.И., Мушинская М.В., Павлова Е.В., Мазеркина Н.А., Румянцев А.Г. Результаты лечения редких эмбриональных опухолей ЦНС у детей. Педиатрия 2008;87(4):64–8. [Kumirova E.V., Zheludkova O.G., Borodina I.D., Gorbatykh S.V., Livshits M.I., Popov V.E., Korshunov A.G., Melikyan A.G., Kushel Yu .V., Ozerova V.I., Ozerov S.S., Gorelyshev S.K., Shcherbenko O.I., Zelinskaya N.I., Polushkina O.B., Govorina E.V., Kholodov B.V., Tarasova E.M., Kuptsova E.V., Privalova L.P., Karelin A.F., Shammasov R.Z., Grishina E.N., Stremneva O.V., Belogurova M.B., Schatz L.I., Mushinskaya M.V., Pavlova E.V., Mazerkina N.A., Rumyantsev A.G. Results of treatment of rare CNS embryonic tumors in children. Pediatriya = Pediatrics 2008;87(4):64–68. (In Russ.)].Желудкова О.Г., Коршунов А.Г., Русанова М.Г., Бородина И.Д., Горбатых С.В., Меликян А.Г., Горелышев С.К., Щербенко О.И., Зелинская Н.И., Озерова В.И. Атипичная тератоид-рабдоидная опухоль центральной нервной системы у детей. Материалы конференции. Сибирский онкологический журнал 2006:45–6. [Zheludkova O.G., Korshunov A.G., Rusanova M.G., Borodina I.D., Gorbatykh S.V., Melikyan A.G., Gorelyshev S.K., Shcherbenko O.I., Zelinskaya N.I., Ozerova V.I. Atypical teratoid-rhabdoid tumor of the central nervous system in children. Conference proceedings. Sibirskiy onkologicheskiy zhurnal = Siberian Oncology Journal 2006:45–6. (In Russ.)].Желудкова О.Г., Коршунов А.Г., Русанова М.Г., Бородина И.Д., Горбатых С.В., Меликян А.Г., Горелышев С.К., Щербенко О.И., Зелинская Н.И., Озерова В.И. Атипичная тератоид-рабдоидная опухоль центральной нервной системы у детей. Материалы конференции. Сибирский онкологический журнал 2006:45–6. [Zheludkova O.G., Korshunov A.G., Rusanova M.G., Borodina I.D., Gorbatykh S.V., Melikyan A.G., Gorelyshev S.K., Shcherbenko O.I., Zelinskaya N.I., Ozerova V.I. Atypical teratoid-rhabdoid tumor of the central nervous system in children. Conference proceedings. Sibirskiy onkologicheskiy zhurnal = Siberian Oncology Journal 2006:45–6. (In Russ.)].Geyer J., Sposto R., Jennings M., Boyett J.M., Axtell R.A., Breiger D., Broxson E., Donahue B., Finlay J.L., Goldwein J.W., Heier L.A., Johnson D., Mazewski C., Miller D.C., Packer R., Puccetti D., Radcliff e J., Tao M.L., Shiminski-Maher T. Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from Children’s Cancer Group. J Clin Oncol 2005;23(30):7621–31. doi: 10.1200/JCO.2005.09.095.Geyer J., Sposto R., Jennings M., Boyett J.M., Axtell R.A., Breiger D., Broxson E., Donahue B., Finlay J.L., Goldwein J.W., Heier L.A., Johnson D., Mazewski C., Miller D.C., Packer R., Puccetti D., Radcliff e J., Tao M.L., Shiminski-Maher T. Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from Children’s Cancer Group. J Clin Oncol 2005;23(30):7621–31. doi: 10.1200/JCO.2005.09.095.Cohen B., Geyer J., Miller D., Curran J.G., Zhou T., Holmes E., Ingles S.A., Dunkel I.J., Hilden J., Packer R.J., Pollack I.F., Gajjar A., Finlay J.L.; Children’s Oncology Group. Pilot Study of Intensive Chemotherapy with Peripheral Hematopoietic Cell Support for Children Less than 3 Years of Age with Malignant Brain Tumors, The CCG-99703 Phase I/II Study. A Report from the Children’s Oncology Group. Pediatr Neurol 2015;53(1):31–46. doi: 10.1016/j.pediatrneurol.2015.03.019.Cohen B., Geyer J., Miller D., Curran J.G., Zhou T., Holmes E., Ingles S.A., Dunkel I.J., Hilden J., Packer R.J., Pollack I.F., Gajjar A., Finlay J.L.; Children’s Oncology Group. Pilot Study of Intensive Chemotherapy with Peripheral Hematopoietic Cell Support for Children Less than 3 Years of Age with Malignant Brain Tumors, The CCG-99703 Phase I/II Study. A Report from the Children’s Oncology Group. Pediatr Neurol 2015;53(1):31–46. doi: 10.1016/j.pediatrneurol.2015.03.019.Combination Chemotherapy, Radiation Therapy, and an Autologous Peripheral Blood Stem Cell Transplant in Treating Young Patients With Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System. https://clinicaltrials.gov/ct2/show/NCT00653068.Combination Chemotherapy, Radiation Therapy, and an Autologous Peripheral Blood Stem Cell Transplant in Treating Young Patients With Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System. https://clinicaltrials.gov/ct2/show/NCT00653068.Fagioli F., Biasin E., Mastrodicasa L., Sandri A., Ferrero I., Berger M., Vassallo E., Madon E. High-dose Thiotepa and Etoposide in Children with poor-prognosis brain tumors. Cancer 2004;100(10):2215–21. doi: 10.1002/cncr.20227.Fagioli F., Biasin E., Mastrodicasa L., Sandri A., Ferrero I., Berger M., Vassallo E., Madon E. High-dose Thiotepa and Etoposide in Children with poor-prognosis brain tumors. Cancer 2004;100(10):2215–21. doi: 10.1002/cncr.20227.Athale U., Duckworth J., Odame I., Barr R. Childhood atypical teratoid rhabdoid tumor of the central nervous system. J Pediatr Hematol Oncol 2009;31(9):651–63. doi: 10.1097/MPH.0b013e3181b258a9.Athale U., Duckworth J., Odame I., Barr R. Childhood atypical teratoid rhabdoid tumor of the central nervous system. J Pediatr Hematol Oncol 2009;31(9):651–63. doi: 10.1097/MPH.0b013e3181b258a9.Duff ner P., Horowitz M.E., Krischer J.P., Friedman H.S., Burger P.C., Cohen M.E., Sanford R.A., Mulhern R.K., James H.E., Freeman C.R., Seidel F.G., Kun L.E. Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 1993;328:1725–31. doi: 10.1056/NEJM199306173282401.Duff ner P., Horowitz M.E., Krischer J.P., Friedman H.S., Burger P.C., Cohen M.E., Sanford R.A., Mulhern R.K., James H.E., Freeman C.R., Seidel F.G., Kun L.E. Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 1993;328:1725–31. doi: 10.1056/NEJM199306173282401.Gardner S., Asgharzadeh A., Green B., Horn B., McCowage G., Finlay J. Intensive induction chemotherapy followed by high dose chemotherapy with autologous hematopoietic progenitor cell rescue in young children newly diagnosed with central nervous system atypical teratoid rhabdoid tumors. Pediatr Blood Cancer 2008;51(2):235–40. doi: 10.1002/pbc.21578.Gardner S., Asgharzadeh A., Green B., Horn B., McCowage G., Finlay J. Intensive induction chemotherapy followed by high dose chemotherapy with autologous hematopoietic progenitor cell rescue in young children newly diagnosed with central nervous system atypical teratoid rhabdoid tumors. Pediatr Blood Cancer 2008;51(2):235–40. doi: 10.1002/pbc.21578.Zaky W., Dhall G., Ji L., Haley K., Allen J., Atlas M., Bertolone S., Cornelius A., Gardner S., Patel R., Pradhan K., Shen V., Thompson S., Torkildson J., Sposto R., Finlay J.L. Intensive induction chemotherapy followed by myeloablative chemotherapy with autologous hematopoietic progenitor cell rescue for young children newly-diagnosed with central nervous system atypical teratoid/rhabdoid tumors: the Head Start III experience. Pediatr Blood Cancer 2014;61(1):95–101. doi: 10.1002/pbc.24648.Zaky W., Dhall G., Ji L., Haley K., Allen J., Atlas M., Bertolone S., Cornelius A., Gardner S., Patel R., Pradhan K., Shen V., Thompson S., Torkildson J., Sposto R., Finlay J.L. Intensive induction chemotherapy followed by myeloablative chemotherapy with autologous hematopoietic progenitor cell rescue for young children newly-diagnosed with central nervous system atypical teratoid/rhabdoid tumors: the Head Start III experience. Pediatr Blood Cancer 2014;61(1):95–101. doi: 10.1002/pbc.24648.Benesch M., Bartelheim K., Fleichhack G., Gruhn B., Schlegel P.G., Witt O., Stachel K.D., Hauch H., Urban C., Quehenberger F., Massimino M., Pietsch T., Hasselblatt M., Giangaspero F., Kordes U., Schneppenheim R., Hauser P., Klingebiel T., Frühwald M.C. High-dose chemotherapy (HDCT) with auto-SCT in children with atypical teratoid/rhabdoid tumors (AT/RT): a report from the European Rhabdoid Registry (EU-RHAB). Bone Marrow Transplant 2014;49(3):370–5. doi: 10.1038/bmt.2013.208.Benesch M., Bartelheim K., Fleichhack G., Gruhn B., Schlegel P.G., Witt O., Stachel K.D., Hauch H., Urban C., Quehenberger F., Massimino M., Pietsch T., Hasselblatt M., Giangaspero F., Kordes U., Schneppenheim R., Hauser P., Klingebiel T., Frühwald M.C. High-dose chemotherapy (HDCT) with auto-SCT in children with atypical teratoid/rhabdoid tumors (AT/RT): a report from the European Rhabdoid Registry (EU-RHAB). Bone Marrow Transplant 2014;49(3):370–5. doi: 10.1038/bmt.2013.208.Maritaz C., Lemare F., Laplanche A., Demirdjian S., Valteau-Couanet D., Dufour C. High-dose thiotepa-related neurotoxicity and the role of tramadol in children. BMC Cancer 2018;18(177):1–9. doi: 10.1186/s12885-018-4090-6.Maritaz C., Lemare F., Laplanche A., Demirdjian S., Valteau-Couanet D., Dufour C. High-dose thiotepa-related neurotoxicity and the role of tramadol in children. BMC Cancer 2018;18(177):1–9. doi: 10.1186/s12885-018-4090-6.Heideman R., Packer R., Reaman G., Allen J.C., Lange B., Horowitz M.E., Steinberg S.M., Gillespie A., Kovnar E.H., Balis F.M. A Phase II Evaluation of Thiotepa in Pediatric Central Nervous System Malignancies. Cancer 1993;72(1):271–5.Heideman R., Packer R., Reaman G., Allen J.C., Lange B., Horowitz M.E., Steinberg S.M., Gillespie A., Kovnar E.H., Balis F.M. A Phase II Evaluation of Thiotepa in Pediatric Central Nervous System Malignancies. Cancer 1993;72(1):271–5.Nicolaides T., Tihan T., Horn B., Biegel J., Prados M., Banerjee A. Highdose chemotherapy and autologous stem cell rescue for atypical teratoid/ rhabdoid tumor of the central nervous system. J Neurooncol 2010;98:117–23. doi: 10.1007/s11060-009-0071-6.Nicolaides T., Tihan T., Horn B., Biegel J., Prados M., Banerjee A. Highdose chemotherapy and autologous stem cell rescue for atypical teratoid/ rhabdoid tumor of the central nervous system. J Neurooncol 2010;98:117–23. doi: 10.1007/s11060-009-0071-6.Fidani P., De Ioris M., Serra A., De Sio L., Ilari I., Cozza R., Boldrini R., Milano G.M., Garrè M.L., Donfrancesco A. A multimodal strategy based on surgery, radiotherapy, ICE regimen and high dose chemotherapy in atypical teratoid/rhabdoid tumours: a single institution experience. J Neurooncol 2009;92(2):177–83. doi: 10.1007/s11060-008-9750-y.Fidani P., De Ioris M., Serra A., De Sio L., Ilari I., Cozza R., Boldrini R., Milano G.M., Garrè M.L., Donfrancesco A. A multimodal strategy based on surgery, radiotherapy, ICE regimen and high dose chemotherapy in atypical teratoid/rhabdoid tumours: a single institution experience. J Neurooncol 2009;92(2):177–83. doi: 10.1007/s11060-008-9750-y.Park E., Sung K., Baek H., Park K.D., Park H.J., Won S.C., Lim D.H., Kim H.S. Tandem High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Young Children with Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System. J Korean Med Sci 2012;27:135–40. doi: 10.3346/jkms.2012.27.2.135.Park E., Sung K., Baek H., Park K.D., Park H.J., Won S.C., Lim D.H., Kim H.S. Tandem High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Young Children with Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System. J Korean Med Sci 2012;27:135–40. doi: 10.3346/jkms.2012.27.2.135.Poplack D., Bleyer W., Horowitz M. Pharmacology of antineoplastic agents in cerebrospinal fl uid. New York: Plenum Press, 1980.Poplack D., Bleyer W., Horowitz M. Pharmacology of antineoplastic agents in cerebrospinal fl uid. New York: Plenum Press, 1980.Kerr J., Berg S., Blaney S. Intrathecal chemotherapy. Crit Rev Oncol Hematol 2001;37(3):227–36. PMID: 11248578.Kerr J., Berg S., Blaney S. Intrathecal chemotherapy. Crit Rev Oncol Hematol 2001;37(3):227–36. PMID: 11248578.Chrzanowska E.K., Bartoszewska J., Drogosiewicz M., Kwiatkowski S., Skowronska-Gardas A. The importance of radiotherapy in paediatric atypical teratoid rhabdoid tumour of the brain. Reports of Practical Oncology & Radiotherapy 2009;14(2):53–7. doi: 10.1016/S1507-1367(10)60022-3.Chrzanowska E.K., Bartoszewska J., Drogosiewicz M., Kwiatkowski S., Skowronska-Gardas A. The importance of radiotherapy in paediatric atypical teratoid rhabdoid tumour of the brain. Reports of Practical Oncology & Radiotherapy 2009;14(2):53–7. doi: 10.1016/S1507-1367(10)60022-3.Seeringer A., Bartelheim K., Kerl K., Hasselblatt M., Leuschner I., Rutkowski S., Timmermann B., Kortmann R.D., Koscielniak E., Schneppenheim R., Warmuth-Metz M., Gerß J., Siebert B., Graf N., Boos J., Frühwald M.C. Feasibility of intensive multimodal therapy in infants aff ected by rhabdoid tumors – experience of the EU-RHAB registry. Klin Padiatr 2014:226:143–8. doi: 10.1055/s-0034-1368719.Seeringer A., Bartelheim K., Kerl K., Hasselblatt M., Leuschner I., Rutkowski S., Timmermann B., Kortmann R.D., Koscielniak E., Schneppenheim R., Warmuth-Metz M., Gerß J., Siebert B., Graf N., Boos J., Frühwald M.C. Feasibility of intensive multimodal therapy in infants aff ected by rhabdoid tumors – experience of the EU-RHAB registry. Klin Padiatr 2014:226:143–8. doi: 10.1055/s-0034-1368719.Spiegler B., Bouff et E., Greenberg M., Rutka J.T., Mabbott D.J. Change in neurocognitive functioning after treatment with cranial radiation in childhood. J Clin Oncol 2004;22(4):706–13. doi: 10.1200/JCO.2004.05.186.Spiegler B., Bouff et E., Greenberg M., Rutka J.T., Mabbott D.J. Change in neurocognitive functioning after treatment with cranial radiation in childhood. J Clin Oncol 2004;22(4):706–13. doi: 10.1200/JCO.2004.05.186.Kralik S.F., Ho C.Y., Finke W., Buchsbaum J.C., Haskins C.P., Shih C.S. Radiation necrosis in pediatric patients with brain tumors treated with proton radiotherapy. AJNR Arn J Neuroradiol 2015;36(8):1572–8. doi: 10.3174/ajnr.A4333.Kralik S.F., Ho C.Y., Finke W., Buchsbaum J.C., Haskins C.P., Shih C.S. Radiation necrosis in pediatric patients with brain tumors treated with proton radiotherapy. AJNR Arn J Neuroradiol 2015;36(8):1572–8. doi: 10.3174/ajnr.A4333.Christopherson K., Rotondo R., Bradley J., Pincus D.W., Wynn T.T., Fort J.A., Morris C.G., Mendenhall N.P., Marcus R.B., Indelicato D.J. Late toxicity following craniospinal radiation for early-stage medulloblastoma. Acta Oncol 2014;53(4):471–80. doi: 10.3109/0284186X.2013.862596.Christopherson K., Rotondo R., Bradley J., Pincus D.W., Wynn T.T., Fort J.A., Morris C.G., Mendenhall N.P., Marcus R.B., Indelicato D.J. Late toxicity following craniospinal radiation for early-stage medulloblastoma. Acta Oncol 2014;53(4):471–80. doi: 10.3109/0284186X.2013.862596.Chiou S., Kao C., Chen Y., Chien C.S., Hung S.C., Lo J.F., Chen Y.J., Ku H.H., Hsu M.T., Wong T.T. Identifi cation of CD133-Positive Radioresistant Cells in Atypical Teratoid/Rhabdoid Tumor. PLoS One 2008;3(5):e2090. doi: 10.1371/journal.pone.0002090.Chiou S., Kao C., Chen Y., Chien C.S., Hung S.C., Lo J.F., Chen Y.J., Ku H.H., Hsu M.T., Wong T.T. Identifi cation of CD133-Positive Radioresistant Cells in Atypical Teratoid/Rhabdoid Tumor. PLoS One 2008;3(5):e2090. doi: 10.1371/journal.pone.0002090.Yock T., Bhat S., Szymonifka J., Yeap B.Y., Delahaye J., Donaldson S.S., MacDonald S.M., Pulsifer M.B., Hill K.S., DeLaney T.F., Ebb D., Huang M., Tarbell N.J., Fisher P.G., Kuhlthau K.A. Quality of life outcomes in proton and photon treated pediatric brain tumors survivors. Radiother Oncol 2014;113(1):89–94. doi: 10.1016/j.radonc.2014.08.017.Yock T., Bhat S., Szymonifka J., Yeap B.Y., Delahaye J., Donaldson S.S., MacDonald S.M., Pulsifer M.B., Hill K.S., DeLaney T.F., Ebb D., Huang M., Tarbell N.J., Fisher P.G., Kuhlthau K.A. Quality of life outcomes in proton and photon treated pediatric brain tumors survivors. Radiother Oncol 2014;113(1):89–94. doi: 10.1016/j.radonc.2014.08.017.De Amorim Bernstein K., Sethi R., Trofi mov A., Zeng C., Fullerton B., Yeap B.Y., Ebb D., Tarbell N.J., Yock T.I., MacDonald S.M. Early clinical outcomes using proton radiation for children with central nervous system atypical teratoid rhabdoid tumors. Int J Radiat Oncol Biol Phys 2013;86(1):114–20. doi: 10.1016/j.ijrobp.2012.12.004.De Amorim Bernstein K., Sethi R., Trofi mov A., Zeng C., Fullerton B., Yeap B.Y., Ebb D., Tarbell N.J., Yock T.I., MacDonald S.M. Early clinical outcomes using proton radiation for children with central nervous system atypical teratoid rhabdoid tumors. Int J Radiat Oncol Biol Phys 2013;86(1):114–20. doi: 10.1016/j.ijrobp.2012.12.004.Davanzo J., Greiner R., Barbour M., Rizk E. Radiation Necrosis Following Proton Beam Therapy in the Pediatric Population: a Case Series. Cureus 2017;9(10):e1785. DOI 10.7759/cureus.1785.Davanzo J., Greiner R., Barbour M., Rizk E. Radiation Necrosis Following Proton Beam Therapy in the Pediatric Population: a Case Series. Cureus 2017;9(10):e1785. DOI 10.7759/cureus.1785.Berland M., Padovani L., Rome A., Pech-Gourg G., Figarella-Branger D., André N. Sustained Complete Response to Metronomic Chemotherapy in a Child with Refractory Atypical Teratoid Rhabdoid Tumor: A Case Report. Front Pharmacol 2017;8:1–4. doi: 10.3389/fphar.2017.00792.Berland M., Padovani L., Rome A., Pech-Gourg G., Figarella-Branger D., André N. Sustained Complete Response to Metronomic Chemotherapy in a Child with Refractory Atypical Teratoid Rhabdoid Tumor: A Case Report. Front Pharmacol 2017;8:1–4. doi: 10.3389/fphar.2017.00792.Wetmore C., Boyett J., Li S., Lin T., Bendel A., Gajjar A., Orr B.A. Alisertib is active as single agent in reccurent atypical teratoid rhabdoid tumors in 4 children. Neuro Oncol 2015;17(6):882–8. doi: 10.1093/neuonc/nov017.Wetmore C., Boyett J., Li S., Lin T., Bendel A., Gajjar A., Orr B.A. Alisertib is active as single agent in reccurent atypical teratoid rhabdoid tumors in 4 children. Neuro Oncol 2015;17(6):882–8. doi: 10.1093/neuonc/nov017.Nemes K., Bens S., Bourdeaut F., Hasselblatt M., Kool M., Johann P., Kordes U., Schneppenheim R., Siebert R., Frühwald M.C. Rhabdoid Tumor Predisposition Syndrome. https://www.ncbi.nlm.nih.gov/books/NBK469816/.Nemes K., Bens S., Bourdeaut F., Hasselblatt M., Kool M., Johann P., Kordes U., Schneppenheim R., Siebert R., Frühwald M.C. Rhabdoid Tumor Predisposition Syndrome. https://www.ncbi.nlm.nih.gov/books/NBK469816/.

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