Chordomas primarily occur in which part of the body?

Chordomas are rare, malignant tumors that arise from remnants of the notochord, a structure that is instrumental in the development of the axial skeleton during fetal development. The primary location for chordomas is the midline regions of the axial skeleton, which encompasses areas such as the base of the skull and the sacrococcygeal region. This midline occurrence is essential to understand, as chordomas are characteristically found along the body's central axis.

Tumors located in the periphery of long bones are typically associated with different types of bone malignancies, and while chordomas can be found in proximity to the vertebral column, they are not known to originate in the peripheral bone structures. Similarly, while the pelvic region may contain chordomas when they emanate from the sacrum, it is the midline of the axial skeleton that is their primary site of occurrence. The cervical spine, while it can be a site for these tumors, is not as commonly affected compared to the sacrococcygeal region and the clivus of the skull, further reinforcing that the axial skeleton in the midline is the most characteristic location for chordomas.