Dedifferentiated chondrosarcoma (DDCS) is a rare and aggressive bone tumor with poor prognosis. Primary DDCS of the mobile spine is extremely rare, particularly in the cervical spine. We herein describe a first case of cervical DDCS in an 81-year-old male presenting with a slowly growing mass. Radiographs showed an expansion of the cortical contour of the C2 lamina and a soft tissue mass with punctate calcification. Magnetic resonance imaging demonstrated a lobulated lesion expanding over the entire lamina and pedicles of C2 with the tumor protuberant to the adjacent soft tissue. A complete tumor resection was performed. Histologically, the majority of the tumor was a low-grade chondrosarcoma component. However, atypical spindle cells that had proliferated in a fascicular pattern with a collagenous stroma, mimicking fibrosarcoma, were focally observed without a transitional zone, and these features confirmed that the tumor was DDCS.
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