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The 2020 World Health Organization classification of bone tumors: what radiologists should know

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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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Funding

National Cancer Center,P30 CA008748

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Authors and Affiliations

  1. Department of Radiology, Memorial Sloan Kettering Cancer Center, 1275 York Ave, New York, NY, 10065, USA

    Sinchun Hwang

  2. Department of Pathology, Memorial Sloan Kettering Cancer Center, 1275 York Ave, New York, NY, 10065, USA

    Meera Hameed

  3. Department of Radiology, Mayo Clinic, 5777 E Mayo Blvd, Phoenix, AZ, 85054, USA

    Mark Kransdorf

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  1. Sinchun Hwang
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  2. Meera Hameed
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  3. Mark Kransdorf
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Correspondence to Sinchun Hwang.

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Key points

• Chondroblastoma, chondromyxoid fibroma, and aneurysmal bone cyst are classified as benign. Osteofibrous dysplasia-like adamantinoma and synovial chondromatosis are now categorized as intermediate (locally aggressive).

• The designation of atypical cartilaginous tumors is reserved for the appendicular skeleton and the histologically identical locally aggressive hyaline cartilage tumor is termed chondrosarcoma grade 1 in the axial skeleton and flat bones.

• Erdheim-Chester Erdheim-Chester disease is no longer considered an intermediate locally aggressive tumor due to its unfavorable clinical outcomes by its multiorgan involvement and is now classified as a hematopoietic neoplasm of bone.

• Ewing sarcoma is no longer classified as a bone tumor and is now addressed in a new category, titled “undifferentiated small round cell sarcomas of bone and soft tissue.”

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Hwang, S., Hameed, M. & Kransdorf, M. The 2020 World Health Organization classification of bone tumors: what radiologists should know. Skeletal Radiol 52, 329–348 (2023). https://doi.org/10.1007/s00256-022-04093-7

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  • DOI: https://doi.org/10.1007/s00256-022-04093-7

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