Chordomas are rare bone tumors that develop in the neuraxis, often in the clivus or sacrococcygeal region. Symptoms depend on the location and treatment involves surgery and radiation therapy, but recurrence and metastasis are common, resulting in a poor prognosis.
A chordoma is a rare type of bone tumor that develops along the spine or at the base of the skull. Chordomas account for less than XNUMX% of all central nervous system tumors and between XNUMX% and XNUMX% of all primary bone tumors. In many cases, chordoma tumors can be treated effectively with surgery, but they have a high recurrence rate and a high risk of metastasis.
Chordomas develop in a region of the body called the neuraxis, so named because of its importance to the central nervous system. During fetal development, the neuraxis contains structures that help regulate the development of this system. In particular, a structure called the notochord is instrumental during the development of the fetal central nervous system. The notochord develops in the spine during fetal growth, but some remnants of notochord cells remain in the body. These residual notochord cells are the location where a chordoma tumor can develop.
Notochord cells typically remain in two locations along the spinal cord. These are the clivus, at the base of the skull, and the sacrococcygeal region, located at the tail end of the spine. For this reason, the clivus and sacrococcygeal region are the two locations where chordomas are most likely to grow. Notochord cells can sometimes remain in other parts of the spine and potentially give rise to chordomas in a location other than the clivus or sacrococcygeal region.
Chordoma symptoms depend on the location of the primary tumor growth. As the tumor grows into the clivus, symptoms typically include headaches and vision disturbances. When the tumor grows in the sacrococcygeal region or elsewhere in the spine, symptoms could include lower back pain and incontinence. If the tumor presses on a nerve, you may experience weakness, pain, or numbness in your legs or arms.
Treatment of chordoma tumors most commonly involves surgery to remove as much of the tumor as possible. This surgery is often followed by radiation therapy to kill any remaining cancer cells. Complete surgical removal of the tumor reduces the risk of recurrence but cannot completely eliminate the risk. Possible complications of cranial surgery include intracranial hemorrhage, meningitis, increased intracranial pressure, and facial palsy. In the case of spinal tumor removal, complications include difficulty walking and bladder or bowel dysfunction.
Although chordomas are very slow growing tumors, they are also very likely to invade surrounding tissue, can metastasize, and can recur even after surgical removal of a primary tumor. For all these reasons, the prognosis for this type of cancer is often poor. A person diagnosed with this type of cancer has an approximately fifty percent chance of surviving five years or more and a ten percent chance of surviving more than ten years.
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