Key result
WHO defines four small round cell sarcoma categories requiring clinical, histologic, and molecular evaluation.
Why the study?
Undifferentiated small round cell sarcomas frequently pose significant diagnostic challenges due to rarity and overlapping morphologic and immunohistochemical findings, requiring molecular testing for definitive diagnosis.
This review provides an overview of the WHO classification and diagnostic features of undifferentiated small round cell sarcomas, which pose significant diagnostic challenges.
Requires molecular testing to avoid misclassifying rare sarcomas in practice; extends WHO framework while leaving therapeutic implications open.
The WHO Classification of Soft Tissue and Bone Tumours currently recognizes four categories of undifferentiated small round cell sarcoma: Ewing sarcoma, round cell sarcoma with EWSR1-non-ETS fusions including NFATc2 and PATZ1, CIC-rearranged sarcoma, and sarcoma with BCOR genetic alterations. These neoplasms frequently pose significant diagnostic challenges due to rarity and overlapping morphologic and immunohistochemical findings. Further, molecular testing, with accompanying pitfalls, may be needed to establish a definitive diagnosis. This review summarizes the clinical, histologic, immunohistochemical, and molecular features of these neoplasms. In addition, differential diagnosis and areas of uncertainty and ongoing investigation are discussed.
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Dehner et al. (2024) conducted a review in Undifferentiated small round cell sarcoma. The WHO classification recognizes four categories of undifferentiated small round cell sarcoma that pose diagnostic challenges requiring clinical, histologic, and molecular evaluation.
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