Chordoma is a rare tumor that usually occurs in the spine and base of the skull. It is a malignant tumor that grows fairly slowly. It can spread to other organs, usually the lungs. It represents only about 1 percent of all malignant bone tumors.

Chordomas develop in a structure called the notochord. The notochord forms the early spine in the beginning stages of fetal development. Most of the notochord is replaced by the spine during the first six months of development. Small areas can remain, and chordomas can form in these areas.

Most chordomas occur at the base of the spine (sacrum), in the tailbone (coccyx) or at the base of the skull (40 percent) but they can occur other places in the spine.

Most patients with chordomas are between 40 and 70 years of age. Occasionally this tumor can occur in younger patients, even children. The average age of people affected is about 55. Chordomas are life-threatening. They can cause death by direct growth or by spreading to other organs. They spread to the lungs about 20-30 percent of the time.

E-cadherin

• expression of chordoma cells suggests a role as a useful diagnostic marker to distinguish chondroid chordoma from chondrosarcoma. PMID: 11964040