Underdown M Jane, Kappa Sarah, Metts Jonathan, Cash Thomas
Department of Pediatrics, Division of Pediatric Hematology/Oncology/BMT, University of Utah Health, Salt Lake City, UT, USA.
Department of Pediatrics, Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Houston, TX, USA.
Hematol Oncol Clin North Am. 2025 Aug;39(4):709-726. doi: 10.1016/j.hoc.2025.04.003. Epub 2025 May 14.
The classification of small round cell sarcomas encompasses a spectrum of diseases ranging from those that are more widely understood, such as Ewing sarcoma and desmoplastic small round cell tumor, to more newly characterized diseases such as capicua (CIC)-rearranged sarcomas, BCOR-altered sarcomas, and sarcomas with EWSR1-non-ETS fusions. This article provides background, unique molecular features, and current and emerging therapies for each round cell sarcoma subtype.
小圆细胞肉瘤的分类涵盖了一系列疾病，从那些较广为人知的疾病，如尤因肉瘤和促纤维组织增生性小圆细胞肿瘤，到更新发现的疾病，如capicua（CIC）重排肉瘤、BCOR改变的肉瘤以及具有EWSR1-非ETS融合的肉瘤。本文提供了每种小圆细胞肉瘤亚型的背景、独特分子特征以及当前和新兴的治疗方法。
