Update on Families of Round Cell Sarcomas Other than Classical Ewing Sarcomas

Abstract

This article focuses on families of round cell sarcomas other than classical Ewing sarcomas. Until recently, these tumors were referred to as so-called Ewing-like tumors, as they morphologically resemble Ewing sarcomas but are negative for canonical fusion transcripts of Ewing sarcomas involving gene members of the ETS family of transcription factors. Clinicopathologic and molecular evidence has dramatically influenced the diagnostic approach of these tumors in recent years. Molecular data that support these sarcoma subtypes are biologically distinct from those of Ewing sarcomas, thereby advocating discarding the all-embracing and confusing terminology of "Ewing-like tumors."

Keywords: Atypical Ewing sarcoma; BCOR; CIC; Ewing sarcoma; Ewing-like sarcoma; Fusion genes; Round cell sarcoma; Sarcoma.