Chordomas are rare tumors that arise from embryonic notochordal remnants along the length of the neuraxis at developmentally active sites. These sites are the ends of the neuraxis and the vertebral bodies. Chordomas constitute less than 1% of CNS tumors and rarely occur in extra-axial locations. Chordomas are thought to arise from ectopic notochord remnants. Chordomas are optimally managed with aggressive surgery, while preserving key structures, and postoperative radiation.
Chordomas are associated with significant morbidity because of their growth patterns and surgical constraints in resection.
An image depicting a chordoma can be seen below.
This pelvic CT scan shows a large presacral mass eroding bone.