HISTOLOGICALLY the chordoma is a benign tumor, but clinically it is destructive. It develops in the residual notochord along the spine and the base of the skull. The majority of cases of chordoma described up to now occurred at the ends of the notochord, ie, in the spheno-occipital region (35%-40%) and in the sacrococcygeal region (45%-50%) of the spine. The remaining 10%-20% of the cases were distributed over other regions of the spine.1
As is known, the nuclei pulposi of the intervertebral discs consist of notochord tissue. The fact that the chordoma develops more frequently in places in which there is no nucleus pulposus suggests that it originates not in organized notochord tissue but in ectopic tissue. Utne and Pugh2 collected 505 cases of chordoma and found that 39% were in the spheno-occipital region, 45% in the sacrococcygeal region, and 16% along the spine. The average age of
SCHINDEL J, MARKOWICZ H. Chordoma Arising From Chordoma Notochord Cells. Arch Otolaryngol. 1966;84(4):441–443. doi:10.1001/archotol.1966.00760030443015
Customize your JAMA Network experience by selecting one or more topics from the list below.