Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children75010.24287/1726-1708-2023-22-4-127-133CLINICAL OBSERVATIONSКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯIntracranial mesenchymal tumors with FET–CREB fusionИнтракраниальные мезенхимальные опухоли со слиянием генов FET–CREBhttps://orcid.org/0000-0002-9722-4590DegtyarevV. A.ДегтяревВ. А.
Russian Federation

Vitaly A. Degtyarev, a pediatric oncologist at the Department of Adolescent  Hematology/Oncology and Neuro-oncology

1 Samory Mashela St., Moscow 117997

Дегтярев Виталий Александрович, врач-детский онколог отделения гематологии/онкологии старшего возраста и нейроонкологии

117997, Москва, ул. Саморы Машела, 1

degtyarev.vitaly@yandex.ruhttps://orcid.org/0000-0002-8580-3499PanferovaA. V.ПанфероваА. В.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0003-2015-5790ZaitsevaM. A.ЗайцеваМ. А.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0003-1308-8622DruyA. E.ДруйА. Е.
Russian Federation

Moscow;

Yekaterinburg

Москва;

Екатеринбург

https://orcid.org/0000-0002-1661-4205SinichenkovaK. Yu.СиниченковаК. Ю.
Russian Federation

Moscow

Москва

MareevaYu. M.МарееваЮ. М.
Russian Federation

Moscow

Москва

RoshchinV. Yu.РощинВ. Ю.
Russian Federation

Moscow

Москва

ArtemovA. V.АртемовА. В.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0001-7750-5216PapushaL. I.ПапушаЛ. И.
Russian Federation

Moscow

Москва

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава РоссииResearch Institute of Medical Cell TechnologiesГАУЗ СО «Институт медицинских клеточных технологий»201220232241271330108202317102023Copyright ©; 2023, «D. Rogachev NMRCPHOI»Copyright ©; 2023, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2023«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/750

Intracranial mesenchymal tumors with rearrangements of the genes of the FET and CREB families were first described in the 2021 World Health Organization classification of tumors of the central nervous system. At the moment, the criteria for the diagnosis and treatment of these tumors have not been unambiguously defined. This article presents two exceptionally rare clinical cases of adolescent patients with IMT with the presence of gene rearrangement of the FET and CREB gene families, who were treated and examined at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. The patients’ parents gave consent to the use of their children's data, including photographs, for research purposes and in publications.

Интракраниальные мезенхимальные опухоли с перестройкой генов семейств FET и CREB впервые были описаны в классификации опухолей центральной нервной системы Всемирной организации здравоохранения 2021 г. На сегодняшний день однозначно не определены критерии диагностики и терапии данного вида образований. В настоящей работе представлено 2 исключительно редких клинических случая пациентов подросткового возраста с интракраниальными мезенхимальными опухолями с наличием перестройки генов семейств FET и CREB, которые проходили лечение и обследование в ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России. Родители пациентов дали согласие на использование информации, в том числе фотографий детей, в научных исследованиях и публикациях.

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