What causes chordoma?
Although first described in 1856, the cause of chordoma is still unknown. In most cases chordomas are sporadic (occur randomly), however several families have been identified with multiple affected family members. This implies that inherited mutations (changes) in a gene or genes can cause some chordomas. Somatic (non-inherited) mutations in that same gene may be responsible for some or all sporadic chordomas. Identifying the gene or genes that cause chordoma will greatly enhance understanding of the disease and could help lead to the development of targeted treatments. Chordomas are thought to arise from remnants of an embryonic rod-shaped structure similar to cartilage called the notochord. During early embryonic development the notochord provides structural rigidity to the embryo and serves as a scaffold for the formation of the spinal column and skull. As the bones of the spine and skull form, the notochord gradually disintegrates, and by birth only remains as scattered cells in th
