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Abstract
Липосаркомы (ЛП) составляют около 20% злокачественных опухолей мягких тканей у взрослых, характеризуются гетерогенностью, частой локализацией в забрюшинном пространстве и на нижних конечностях, а также низкой чувствительностью к лучевой терапии. По классификации ВОЗ выделяют три подтипа: WDL/DDL, MRCL и PLS. Для WDL/DDL (40–45%) типичны амплификации 12q13–15 с гиперэкспрессией MDM2, CDK4, HMGA2 и FRS2; дедифференцировка связана с коамплификацией JUN и ASK1/MAP3K5 и снижением C/EBPα и Klotho. MRCL характеризуются транслокациями t(12;16) (FUS-DDIT3) и t(12;22) (EWSR1-DDIT3), активацией путей PI3K/Akt и SRC/FAK/RHO/ROCK, а их прогрессия обусловлена инактивацией DLK-DIO3. Плеоморфные ЛП (<5%) отличаются высокой злокачественностью, сложными кариотипами, мутациями TP53, RB1, NF1 и гиперэкспрессией VEGF, сурвивина и Bcl-2. Эти молекулярные изменения определяют диагностику, прогноз и возможности таргетной терапии.
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