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Undifferentiated Round Cell Sarcomas: A Comprehensive Review and Practical Diagnostic Guide with an Emphasis on
Paige O'Connor1, Isidro Machado2,3,4,5, Jeffrey M Cloutier1
1Department of Pathology and Laboratory Medicine, Dartmouth Hitchcock Medical Center, Lebanon, NH, USA.
International Journal of Surgical Pathology
|April 1, 2026
Summary
Undifferentiated round cell sarcomas are rare skin tumors that are hard to diagnose. This review details their features and diagnostic methods to improve classification and treatment.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Undifferentiated round cell sarcomas are rare malignant tumors.
- Superficial presentations are difficult to diagnose, mimicking other cancers.
- Molecular diagnostics have refined classification into distinct entities.
Purpose of the Study:
- To provide an overview of undifferentiated round cell sarcomas.
- To emphasize clinical, histologic, immunohistochemical, and molecular features.
- To outline mimics at superficial sites and propose a diagnostic framework.
Main Methods:
- Review of clinical, histologic, immunohistochemical, and molecular features.
- Discussion of diagnostic mimics in superficial locations.
- Highlighting an integrated diagnostic framework.
Main Results:
- These tumors present diagnostic challenges due to overlapping morphology and immunophenotypes.
- WHO recognizes distinct entities like Ewing sarcoma, CIC-rearranged, BCOR-altered, and EWSR1-non-ETS fusion sarcomas.
- Superficial presentations are prone to misclassification.
Conclusions:
- Accurate recognition of undifferentiated round cell sarcomas is crucial.
- An integrated diagnostic approach combining morphology, immunohistochemistry, and molecular testing is essential.
- Correct classification directly impacts prognosis and therapeutic decisions.
