As a congenital condition (present at birth), the causes of chordoma appear to have a genetic origin. However, in some cases the cause is not known.
The condition is tied to the ‘T’ gene, which controls the making of the brachyury protein. Brachuyry plays an important role in development of the notochord, a flexible structure in the back that eventually disappears to be replaced by the bones of the spinal column prior to birth. If the ‘T’ gene is duplicated or abnormal, it may result in over production of the brachuyry protein. In some cases, a few of the notochord cells remain and may grow abnormally resulting in a chordoma. The altered ‘T’ gene is autosomal dominant, which means it can be inherited from one parent.