Abstract

Improved understanding of tumor biology through molecular alteration and genetic advances has resulted in a number of major changes in the 2020 World Health Organization's (WHO) classification of bone tumors. These changes include the reclassification of the existing tumors and the introduction of several new entities. A new chapter on undifferentiated small round cell sarcomas of bone and soft tissue was added to classify Ewing sarcoma and the family of Ewing-like sarcomas, which share similar histologies but different molecular and clinical behaviors. Knowledge of the current classification of bone tumors is essential to ensure the appropriate recognition of the inherent biological potential of individual osseous lesions for optimal treatment, follow-up, and overall outcome. This article reviews the major changes to the 2020 WHO's classification of primary bone tumors and the pertinent imaging of selected tumors to highlight these changes.

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