A chordoma is a rare cancerous (malignant) primary bone tumor that usually occurs along the spine or where the skull sits atop the spine (skull base). This type of tumor most often occurs at the skull base, spine or bottom of the spine (sacrum). It forms from small remnants of a coil of cells in the embryo that develops into the disks of the spinal column.
A chordoma is found in men twice as often as in women, with most tumors occurring between ages 50 and 70, although can be seen at any age.
Though commonly slow growing, a chordoma is a difficult tumor to treat because it's near the spinal cord or other critical structures, such as the carotid artery and brain tissue.
Tests and procedures used to diagnose a chordoma include:
Removing a sample of cells for laboratory testing (biopsy). A biopsy is a procedure to remove a sample of suspicious cells for laboratory testing. In the lab, specially trained doctors called pathologists examine the cells under microscopes to determine whether cancer cells are present.
It is important to seek the opinion of a doctor experienced with diagnosing and treating chordomas. The person performing your biopsy will ideally consult with experienced surgeons to plan the procedure so that it can be done in a way that won't interfere with a later operation.
- Obtaining more detailed imaging. Your doctor may recommend imaging tests to help visualize your chordoma and determine whether it has spread beyond the spine or skull base. Tests may include an MRI or CT scan.
After you receive a diagnosis of chordoma, your doctor will develop a treatment plan tailored to your needs in consultation with an expert in cancer and radiation therapy (radiation oncologist) and a surgical oncologist.
Chordoma treatment depends on the size and its location as well as whether it has invaded nerves or other tissue. Options may include surgery, radiation therapy — including proton therapy — stereotactic radiosurgery, chemotherapy and targeted therapies. Tumors may recur after treatment.
If you decide to undergo chordoma surgery, ask about your doctor's experience with complex cranial or spinal surgery. This type of surgery results in fewer complications when done by highly experienced, multidisciplinary surgical teams with expertise in chordomas.
Treatment for a sacral spine tumor
- Surgery. The goal of surgery for a sacral spine tumor is usually to remove the entire tumor in one piece, if possible. Surgery may be difficult to perform because the tumor is near critical structures in the spinal cord.
Radiation therapy. Radiation therapy uses high-energy beams, such as X-rays or protons, 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 your body. Radiation therapy may be used before or after surgery or if surgery isn't an option.
Treatment with newer types of radiation treatment, such as proton therapy, allows doctors to use higher doses of radiation while protecting healthy tissue, which may be more effective in treating a chordoma.
- Radiosurgery. Stereotactic radiosurgery uses multiple beams of radiation to kill the cancer cells in a very small area. Each beam of radiation isn't very powerful, but the point where all the beams meet — at the chordoma — receives a large dose of radiation to kill the cancer cells.
- Other treatments. Sometimes chemotherapy and targeted drug therapy are used to treat a chordoma.
Treatment for skull base tumor
Treatment for a skull base tumor usually involves surgery followed by radiation therapy. The goal of surgery is to remove as much of the tumor as possible without harming nearby healthy tissue or causing undue new problems. Complete resection may not be an option if it's near critical structures, such as the carotid artery. Endoscopic surgery as well as traditional approaches may be needed or used together to remove as much of the tumor as possible at the lowest risk possible.
Surgery is usually followed by radiation therapy to kill any remaining cancer cells and help prevent recurrence.