Chordoma is a rare cancerous (malignant) primary bone tumor that usually occurs along the spine. Chordoma most often occurs in the base of the spine (sacrum) or in the base of the skull.
Chordomas form from small remnants of the notochord, a coil of cells in the embryo that develops into the spinal cord.
A chordoma may occur at any age but is most common in adults. Although slow growing, chordomas are difficult tumors to treat because of their close proximity to the spinal cord.
Symptoms
The most common signs of chordoma are pain and neurological changes. Skull base chordomas most often cause headache, neck pain or double vision. If large enough, they may affect facial sensation or movement, voice, speech, and swallowing function. Chordomas of the spine and sacrum can cause changes in bowel and/or bladder function, pain, aching, tingling, numbness, or weakness of the arms and legs. Often sacral chordomas do not cause symptoms until the tumor is quite large and sometimes a lump is the first sign.
Causes
All cancers are the result of mutations in a cell’s DNA, which cause the cell to acquire harmful properties such as the ability to divide infinitely, to invade other tissues, and to avoid destruction by the immune system.
Like most cancers, almost all chordomas are sporadic, meaning that they develop as a result of mutations that occur at random during the course of one’s life.
There are no specific environmental factors that have been linked to chordoma, although the environment contributes to sporadic mutations.
A very small fraction of chordomas are hereditary (familial), meaning that they are caused by mutations passed on from one’s parents.
Risk factors
There are no known environmental, dietary or lifestyle risk factors.
Complications
The list of complications that have been mentioned in various sources for chordoma includes a primary brain neoplasm and back pain.
Treatment
Treatment for chordoma may include:
- Surgery. The primary treatment for chordoma is surgery, although it may be difficult to perform because the tumor is near critical structures in the brain and spinal cord. The goal of surgery is to remove the entire tumor in one piece, whenever possible. Surgery for chordoma is often complex and requires high professionals.
Radiation therapy uses high-energy beams, such as X-rays, to kill cancer cells. During radiation therapy, you lie on a table as a machine moves around you, directing the radiation beams to precise points on the body. Radiation therapy may be used before or after surgery or in cases when surgery isn't an option.
- Radiosurgery. Stereotactic radiosurgery uses multiple beams of radiation to kill the cancer cells in a very small area. Each beam of radiation isn't particularly powerful, but the point where all the beams meet at the chordoma receives a very large dose of radiation to kill the cancer cells.
Prevention
At this time, no means exists for preventing chardoma. However, researchers continue to investigate hereditary causes and the reasons behind chardoma in people with no apparent genetic links.
