Chordomas are slow-growing malignant bone tumors that can occur anywhere along the spinal column, from the skull base to the tail bone. Chordomas are rare, accounting for only 1 percent of cancers of the central nervous system, but they are considered aggressive and have a high recurrence rate.
Chordomas are thought to develop from remnants of the notochord, which is embryonic tissue. During fetal development, most of the notochord is replaced with the spinal cord. Sometimes, however, small amounts of the notochord remain, and, very rarely, these remnants can develop into a chordoma.
A chordoma can occur at any age, although it is most commonly diagnosed in adults between 40 and 70. Chordomas, especially the types that appear at the bottom of the spine, affect more women than men. Childhood chordomas are extremely rare. Skull base chordomas are more common in younger patients; spinal chordomas are more likely after age 50.
Different segments of the spine correlate with different parts of the body, and so a chordoma’s effects depend on where along the spine it occurs. (See Symptoms of a Chordoma.)
Chordomas can be grouped into different types by looking at a sample under a microscope. The three forms are conventional/classic, chondroid, and dedifferentiated.
Reviewed by: Rohan Ramakrishna, MD
Last reviewed/last updated: December 2024
Illustration by Thom Graves CMI