tumorsMalignant tumoursMalignant tumours2224-50572587-6813Rosoncoweb10.18027/2224-5057-2024-14-3s1-35-41tumors-1345Research ArticleСтатьиАгрессивный фиброматозhttps://orcid.org/0000-0002-4118-9642ВладимироваЛ. Ю.

Любовь Юрьевна Владимирова

Ростов-на-Дону, ул. 14 Линия, 63

rnioi@list.ruhttps://orcid.org/0009-0008-2374-9133АлькинаА. К.

Алькина Анна Константиновна

Ростов-на-Дону, ул. 14 Линия, 63

ФГБУ «НМИЦ онкологии» Минздрава РоссииRussian Federation202418112024143s13541Copyright © Владимирова Л.Ю., Алькина А.К., 20242024Владимирова Л.Ю., Алькина А.К.Владимирова Л.Ю., Алькина А.К.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.malignanttumors.org/jour/article/view/1345

Агрессивный фиброматоз (АФ) или десмоидные опухоли (ДО) — это локально агрессивные и инвазивные опухоли мягких тканей мезенхимального происхождения, которые сложно лечить из‑за их вариабельных клинических проявлений, непредсказуемого клинического течения и отсутствия в настоящее время одобренных методов лечения. Это группа ЗНО редко встречается в клинической практике онколога, в связи с чем диагностика и опции лечения данной патологии существенно ограничены. На сегодняшний день, нет стандартного подхода к ведению больных с АФ, поэтому в зависимости от локализации опухоли, ее симптомов и риска развития осложнений и прогрессирования подбирается один из оптимальных методов лечения: хирургическое вмешательство или другие локальные методы, системная терапия или динамическое наблюдение. Лекарственная терапия активно развивается на протяжении последних десятилетий: от химиотерапии до внедрения тирозинкиназных ингибиторов (ТКИ) и одобрения новых молекул. В настоящее время сорафениб и пазопаниб являются одними из наиболее используемых ТКИ. Кроме того, новые ингибиторы гамма‑секретазы (нирогацестат и AL102) обещают быть эффективными средствами для лечения АФ с управляемым профилем безопасности.

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