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<article 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" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="other" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Supportive Therapy in Oncology</journal-id><journal-title-group><journal-title xml:lang="en">Supportive Therapy in Oncology</journal-title><trans-title-group xml:lang="ru"><trans-title>Поддерживающая терапия в онкологии</trans-title></trans-title-group></journal-title-group><issn publication-format="print">3034-2473</issn><issn publication-format="electronic">3034-3178</issn><publisher><publisher-name xml:lang="en">Publishing House ABV Press</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">51</article-id><article-id pub-id-type="doi">10.17650/3034-2473-2025-2-1-30-43</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>REVIEWS</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>ОБЗОРНЫЕ СТАТЬИ</subject></subj-group><subj-group subj-group-type="article-type"><subject></subject></subj-group></article-categories><title-group><article-title xml:lang="en">Management of selumetinib-associated toxicity in children with neurofibromatosis type 1 and plexiform neurofibromas</article-title><trans-title-group xml:lang="ru"><trans-title>Управление токсичностью, ассоциированной с применением селуметиниба у детей с нейрофиброматозом 1-го типа и плексиформными нейрофибромами</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2003-0982</contrib-id><name-alternatives><name xml:lang="en"><surname>Dinikina</surname><given-names>Yu. V.</given-names></name><name xml:lang="ru"><surname>Диникина</surname><given-names>Ю. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Yulia Valerevna Dinikina  </p><p>2 Akkuratova St., Saint Petersburg 197341 </p></bio><bio xml:lang="ru"><p>Юлия Валерьевна Диникина </p><p>197341 Санкт-Петербург, ул. Аккуратова, 2 </p></bio><email>dinikina_yuv@almazovcentre.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6547-0925</contrib-id><name-alternatives><name xml:lang="en"><surname>Dekhtyareva</surname><given-names>N. S.</given-names></name><name xml:lang="ru"><surname>Дехтярева</surname><given-names>Н. С.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>2 Akkuratova St., Saint Petersburg 197341 </p></bio><bio xml:lang="ru"><p>197341 Санкт-Петербург, ул. Аккуратова, 2 </p></bio><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Almazov National Medical Research Center, Ministry of Health of Russia</institution></aff><aff><institution xml:lang="ru">ФГБУ «Национальный медицинский исследовательский центр им. В.А. Алмазова»</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2025-04-15" publication-format="electronic"><day>15</day><month>04</month><year>2025</year></pub-date><volume>2</volume><issue>1</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>30</fpage><lpage>43</lpage><history><date date-type="received" iso-8601-date="2025-04-07"><day>07</day><month>04</month><year>2025</year></date><date date-type="accepted" iso-8601-date="2025-04-07"><day>07</day><month>04</month><year>2025</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2025, Dinikina Y.V., Dekhtyareva N.S.</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2025, Диникина Ю.В., Дехтярева Н.С.</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="en">Dinikina Y.V., Dekhtyareva N.S.</copyright-holder><copyright-holder xml:lang="ru">Диникина Ю.В., Дехтярева Н.С.</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://stio.abvpress.ru/jour/article/view/51">https://stio.abvpress.ru/jour/article/view/51</self-uri><abstract xml:lang="en"><p>According to the statistics of major studies, neurofibromatosis is one of the most common genetic diseases associated with tumor syndrome with incidence rate of 1 case per 3000–6000 people. The most common benign neoplasms typical for neurofibromatosis type 1 are plexiform and cutaneous neurofibromas which amount to up to 95 % of all NF1-associated benign tumors.Selumetinib is a selective mitogen-activated protein kinase types 1 and 2 inhibitor which showed promising results in the treatment of inoperable symptomatic plexiform neurofibromas. Objective response rate for selumetinib was 68 %. In 2021, selumetinib was registered in the Russian Federation and included into the State Register of Medicinal Remedies. It is important to consider that development of clinically significant adverse events during this therapy can cause drug discontinuation, reduce patients’ adherence to therapy and as a result negatively affect the overall treatment effectiveness.The article presents a review of adverse events of selumetinib, their prevention and treatment options based on the current international guidelines.</p></abstract><trans-abstract xml:lang="ru"><p>Согласно статистическим данным крупных исследований, нейрофиброматоз является одним из наиболее распространенных генетических заболеваний, ассоциированных с опухолевым синдромом, встречаемость которого среди населения составляет 1/3000–1/6000 случаев. Среди доброкачественных новообразований, характерных для нейрофиброматоза 1-го типа, наиболее часто встречаются кожные и плексиформные нейрофибромы, составляя в совокупности до 95 % всех доброкачественных опухолей, ассоциированных с данным заболеванием.Многообещающие результаты в лечении неоперабельных симптоматических плексиформных нейрофибром продемонстрировал препарат селуметиниб – селективный ингибитор митогенактивируемой протеинкиназы 1-го и 2-го типов. Общая доля пациентов с объективным ответом достигла 68 %. В 2021 г. препарат прошел государственную регистрацию и включен в государственный реестр лекарственных средств. Развитие клинически значимых нежелательных явлений на фоне специфической терапии может являться причиной отмены селуметиниба, снизить приверженность пациентов к терапии и, как следствие, отрицательно влиять на общую эффективность лечения. Представлен обзор профиля нежелательных явлений селуметиниба, методов их профилактики и лечения на основании существующих международных рекомендаций.</p></trans-abstract><kwd-group xml:lang="en"><kwd>neurofibromatosis type 1</kwd><kwd>plexiform neurofibroma</kwd><kwd>targeted therapy</kwd><kwd>toxicity</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>нейрофиброматоз 1-го типа</kwd><kwd>плексиформная нейрофиброма</kwd><kwd>таргетная терапия</kwd><kwd>токсичность</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Boyd K.P., Korf B.R., Theos A. Neurofibromatosis type 1. J Am Acad Dermatol 2009;61(1):1–14. DOI: 10.1016/j.jaad.2008.12.051</mixed-citation></ref><ref id="B2"><label>2.</label><mixed-citation>Choi J., An S., Lim S.Y. Current concepts of neurofibromatosis type 1: pathophysiology and treatment. Arch Craniofac Surg 2022;23(1):6–16. 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