CTICasesBCOR Sarcoma of the Anterior Mediastinum
Imaging findings
Chest CT reveals a large, solid, inhomogeneous anterior mediastinal mass that compresses adjacent mediastinal structures. Biopsy and subsequent surgical resection demonstrate a BCOR-rearranged sarcoma, characterized as an undifferentiated small round cell sarcoma.
Key takeaways
BCOR sarcomas are a newly recognized group of undifferentiated small round cell sarcomas that historically overlapped with or were classified as Ewing sarcomas. They are characterized by specific BCOR gene rearrangements, can occur in bone or soft tissues, exhibit rapid growth, and are currently managed with treatment protocols similar to Ewing sarcoma.
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