Undifferentiated Small Round Cell Sarcomas of Bone

Abstract

Undifferentiated small round cell sarcomas represent a heterogeneous group of mesenchymal neoplasms. While imprecise, this term nevertheless provides a useful framework for conceptualizing these tumors. This article highlights current trends in their classification based on morphology, immunohistochemistry, and advanced molecular techniques. As next-generation sequencing becomes commonplace in diagnostic laboratories pathologists can expect to differentiate these tumors with increasing confidence, and actively contribute to related discoveries. Ultimately, when synthesized with rigorous clinical outcome data and other investigative techniques, a more robust landscape for the molecular diagnosis and classification of undifferentiated small round cell sarcomas is expected to emerge in the future.

Keywords: BCOR-associated sarcoma; CIC-associated sarcoma; ETS; Ewing family tumor; FET; Undifferentiated round cell sarcoma.