Chordoma is a rare, slow growing malignant tumor that develops in the spine and skull bones. It is thought to form from the remnants of the notochord (a preliminary structure present in a developing baby in the womb, which eventually forms the spinal cord). The exact cause of a chordoma has not been established with certainty.
Chordomas are more common in adults than children. They can be life-threatening and difficult to treat and therefore require specialized follow-up care.
The most evident signs of a chordoma are pain and neurological changes. Symptoms typical to a skull-based chordoma include headache, issues with vision such as double vision, and pain in the neck region. Symptoms vary depending on the size of the tumor. For instance, a large chordoma may bring about changes in voice and speech, as well as changes in facial sensation or movement and swallowing function. Some other symptoms include weakness in the limbs, pain, body pain, and changes in the functioning of the bowel or the bladder.
Diagnosis involves imaging tests such as CT or MRI scans. Your doctor may recommend X-rays to determine the extent of bone damage due to the tumor.
Treatment of a chordoma involves removing the maximum possible amount of tumor tissue via surgery. Surgery is followed by an aggressive dose of radiation therapy to kill any surrounding tumor cells. Radiation therapy is usually recommended as it reduces the risk of recurrence and prolongs survival. In cases of advanced chordoma, chemotherapy may slow down the growth of the tumor or temporarily arrest the progression of the disease. Molecularly targeted cancer drugs are also gaining popularity as a treatment approach for chordomas.