A chordoma is a type of cancer that occurs at the base of the skull or along the spinal column. Chordomas are commonly treated by neurologists and neurosurgeons because they occur in and around the bony tissues surrounding the central nervous system. However, they are not true cancers of the brain and spinal cord, as they do not arise from neurons or the glial cells that support neurons. Rather, they are categorized as sarcomas—tumors of cartilage, bone, and other connective tissues.
Chordomas get their name because they are thought to arise from remnants of the notochord. The notochord is a primitive, cartilage-like structure that is a precursor and scaffold for the developing spinal column in embryonic humans. As the embryo grows, the notochord eventually develops into the nucleus pulposus—the center of the intervertebral discs that separate and cushion the individual vertebrae of your spine. It is thought that, for reasons not known at this time, remnant chordoma cells persist in some people, and in some cases, these cells give rise to chordoma tumors.
How common are chordomas?
Chordomas are rare. Roughly 300 cases per year are diagnosed in the United States. They are slightly more common in men.
Who gets chordomas?
Chordomas of the skull base are more common in young people, while chordomas of the lower spine are more common later in life. However, chordomas can occur at any location on the spine or skull base at any age.
How are chordomas diagnosed?
Diagnostic imaging such as magnetic resonance imaging (MRI) and computed tomography (CT) can alert doctors to abnormal structures in and around your brain and spinal cord.
Definitive diagnosis of a chordoma is dependent on biopsy and examination of the tumor tissue by a neuropathologist.
Neurological problems and pain are often the first symptoms associated with chordomas, but the size and location of the tumor dictate the presence and severity of symptoms.
For example, a chordoma occurring at the base of the skull might produce headaches of gradually increasing severity as the tumor grows, while a chordoma at the base of the spine might not produce any symptoms until the cancerous mass is large and forms a noticeable lump.
As a rule of thumb, it is best to contact your physician if you notice persistent pain or neurological problems, as these could be signs of a number of different conditions. Your doctor will be able to perform diagnostic tests to isolate the cause of your problems and suggest treatments that may help.
Surgical removal is the optimal and standard treatment for chordomas. Your doctor will try to remove as much of your tumor as possible. Surgical removal of your chordoma can be complicated by factors like large size or involvement of surrounding nerves, arteries and veins, bones, and organs.
Radiation therapy such as Gamma Knife and Cyberknife can be used after surgical removal to delay recurrence of the tumor and increase survival time.
Chemotherapy is less effective for chordomas than for other types of cancer.
- Date of last review: January 24, 2017