- Last Updated: May 30, 2017
Chordomas are a rare cancer that are related to the sarcoid tumors and occur at a rate of 1 in 125,000. There are roughly 2,400 people with chordomas in the United States, and 3,600 in Europe. Three hundred people in the US are diagnosed with chordomas each year, and they are the most common tumor of the sacrum and cervical spine.
Pain and neurologic changes are the classic signs of chordomas. Depending on the location, headache, double vision or neck pain are common, and some are able to get large enough to impact facial sensation, speech and swallowing function. In the sacral portion of the spine, one can see changes in bowel or bladder function, pain, tingling, numbness or weakness. Sometimes the tumors can get so large that the first sign is a noticeable lump.
The overall survival for chordoma patients is 7 years, with a survival breakdown as follows: 5 years (68%), 10 years (40%), and 20 years (13%).
The current treatment is surgically based, with the goal being to remove as much of the chordoma as is possible without doing unacceptable harm. If it is possible to remove the entire chordoma at the first surgery, a cure is possible, but it is very unlikely. Eventually the chordoma is likely to return at or near the initial spot. Radiation therapy has been used to help delay reoccurrence. Chemotherapy has been mostly ineffective, as the tumors grow very slowly.
Focused ultrasound has been used to treat four patients with sacral chordomas in England with encouraging results via focused ultrasound that was guided by ultrasound. The researchers report tumor volume was reduced over time in three patients for whom follow up scans were available. Tumor necrosis was documented in two out of the three patients. The researchers have indicated that they plan to open a national trial for sacral chordoma with long term follow up.
Gillies MJ, Lyon PC, Wu F, Leslie T, Chung DY, Gleeson F, Cranston D, Bojanic S. High-intensity focused ultrasonic ablation of sacral chordoma is feasible: a series of four cases and details of a national clinical trial. Br J Neurosurg. 2016 Dec 12:1-6.