Ewing Sarcoma, Desmoplastic Small Round Cell Tumor, and Other Round Cell Sarcomas

Hematol Oncol Clin North Am. 2025 Aug;39(4):709-726. doi: 10.1016/j.hoc.2025.04.003. Epub 2025 May 14.

Abstract

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.

Keywords: BCOR; CIC-DUX4; Desmoplastic small round cell tumor; EWSR1; Ewing sarcoma; Ewing-like sarcoma; Round cell sarcomas; Small round blue cell tumor.

Publication types

  • Review

MeSH terms

  • Bone Neoplasms* / diagnosis
  • Bone Neoplasms* / genetics
  • Bone Neoplasms* / therapy
  • Desmoplastic Small Round Cell Tumor* / diagnosis
  • Desmoplastic Small Round Cell Tumor* / genetics
  • Desmoplastic Small Round Cell Tumor* / metabolism
  • Desmoplastic Small Round Cell Tumor* / therapy
  • Humans
  • Oncogene Proteins, Fusion / genetics
  • RNA-Binding Protein EWS / genetics
  • Sarcoma, Ewing* / diagnosis
  • Sarcoma, Ewing* / genetics
  • Sarcoma, Ewing* / metabolism
  • Sarcoma, Ewing* / pathology
  • Sarcoma, Ewing* / therapy
  • Sarcoma, Small Cell* / diagnosis
  • Sarcoma, Small Cell* / genetics
  • Sarcoma, Small Cell* / therapy

Substances

  • RNA-Binding Protein EWS
  • EWSR1 protein, human
  • Oncogene Proteins, Fusion