Carter Cody S, Patel Rajiv M
Department of Pathology, Michigan Medicine, University of Michigan, 2800 Plymouth Road, Building 35, Ann Arbor, MI 48109, USA.
Department of Pathology, Sections of Dermatopathology and Bone and Soft Tissue Pathology, Michigan Medicine, University of Michigan, 2800 Plymouth Road, Building 35, Ann Arbor, MI 48109, USA; Department of Dermatology, Michigan Medicine, University of Michigan, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA.
Surg Pathol Clin. 2019 Mar;12(1):191-215. doi: 10.1016/j.path.2018.10.008. Epub 2018 Dec 4.
Round cell sarcomas morphologically similar to Ewing sarcoma, but lacking the classic immunohistochemical features, EWSR-ETS family fusions, and other signs of differentiation, are classified as Ewing-like sarcomas. Recent molecular advances led to the discovery and characterization of two recurrent oncogenic fusion rearrangements, CIC-DUX4 and BCOR-CCNB3, in a significant subset of Ewing-like sarcomas. Uncovered alternate fusion partners broadened the proposed classification of these tumors to CIC-rearranged sarcomas and BCOR-rearranged sarcomas. This article summarizes the clinicopathologic and molecular features of these entities, with particular attention paid to those features that overlap with and distinguish these sarcomas from other round cell sarcomas.
形态学上与尤因肉瘤相似，但缺乏经典免疫组化特征、EWSR-ETS家族融合及其他分化迹象的圆形细胞肉瘤，被归类为尤因样肉瘤。最近的分子研究进展导致在相当一部分尤因样肉瘤中发现并鉴定出两种复发性致癌融合重排，即CIC-DUX4和BCOR-CCNB3。发现的其他融合伙伴将这些肿瘤的拟分类扩展为CIC重排肉瘤和BCOR重排肉瘤。本文总结了这些实体的临床病理和分子特征，特别关注那些与其他圆形细胞肉瘤重叠以及区分这些肉瘤的特征。
