Chordomas are unusual tumors with seemingly unconventional traits. Most notably, they are malignant primary bone tumors but not sarcomas. They derive from embryologic cell remnants but predominantly affect late-middle-age adults, and they behave aggressively but grow slowly. Chordomas present with long-standing symptoms related to local mass effect or invasion. At the skull base, they often cause headaches and cranial neuropathies. In the spine, they incite low-grade pain and either weakness, numbness, constipation, or incontinence. Imaging interpreters play an important role in initial diagnosis, treatment response, and surveillance for recurrence.

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