Chordoma is a rare tumor (about 300 cases diagnosed in the US/year) that develops in the spine and the skull base. Chordoma arises from the notochord. The notochord is fetal tissue that forms the basis of the spinal cord. The notochord is eventually incorporated into the developed vertebral column as the centers of the vertebral discs.
Some notochordal cells remain behind. In rare occasions these cells can become chordoma. Occasionally chorodomas may form in different areas of the spine at the same time. Chordoma has the tendency to recur especially if not treated correctly. It may also spread to other parts of the body especially the liver and the lungs, though this usually occurs later in the disease process.
There are 4 types of chordoma. Conventional is the most common followed by Chondroid. There is little difference in the course of the disease between these 2 types. Poorly differentiated and Dedifferentiated tend to be more aggressive. Poorly differentiated chordoma is more common in younger people and children. They may respond to conventional chemotherapy where conventional and chondroid chordoma do not.
Symptoms of chordoma differ depending on which part of the spine they occur in. The most common symptoms for skull based tumors are blurred vision or double vision and headache. Chordoma of the sacrum may cause lower back pain or changes in bowel or bladder function. Symptoms caused by chordoma of the mobile spine will depend on where the tumor is located and what nerves it is compressing.
Chordoma is best seen on MRI but can also be visualized with a CT scan. Chordoma cannot be seen on x-ray.
Although chordoma does have a distinctive appearance on MRI Benign Notochordal Cell Tumors (BNCT) and Chondrosarcoma may appear similar. The only way to confirm the diagnosis is to perform a biopsy. This can be safely done in suspected sacral and mobile spine chordoma by CT guided needle biopsy. In clival (skull base) chordoma biopsy can be difficult and the surgeon may decide to remove the tumor.
Open biopsy, where the surgeon opens the skin and removes a piece of the tumor for testing should never be done. During an open biopsy tumor cells will likely spill contaminating the wound, allowing the chordoma to seed and decreasing your chance for a good outcome.
The primary treatment for chordoma is en-bloc, (removal of the tumor in one piece) resection. Because of their location en-bloc resection is usually not possible with skull based tumors. Is essential that a team of experienced surgeons remove the tumor.
Surgery may be combined with radiation before or after the surgery to improve the chance of a successful outcome.
With the exception of poorly differentiated and dedifferentiated chemotherapy does not have a role in treating primary chordoma. Some studies have indicated that targeted chemotherapy agents may have a role in slowing the disease progression in recurrent and metastatic chordoma. Research is currently underway to develop more effective chemotherapy agents.
Things to Remember:
As long as the tumor is not severely compressing the spinal cord you have time to consider your options. Chordoma grows and spreads very slowly. Do Not rush into treatment.
Always consult with a surgeon who is very experienced in the care and treatment of chordoma even if you have to travel. There are resources available to help you get the correct treatment.