What is a chordoma?
A chordoma is a very rare type of malignant bone tumor that develops in the bones of the skull or the spine. It arises from embryologic remnant cells from the notochord, which are the cells present in the developing embryo that eventually form the spinal column and more specifically the center of the shock-absorbing discs.
This type of tumor is usually located deep in the spinal column or skull base and is close to critical nerves and blood vessels, making them challenging to treat. Men are slightly more commonly affected than women.
Chordomas are typically low-grade and slow-growing but can be locally aggressive and invasive and may cause fracture or pressure on critical neurologic structures. They typically affect people ages 40-60 but have been reported in children and the elderly.
About 50% of chordomas are found at the base of the spine below the lumbar vertebrae in a bone called the sacrum, 35% occur in the skull base, and 15% occur in the vertebrae of the cervical, thoracic, or lumbar vertebrae. With appropriate treatment, many patients with a chordoma will be cured, and the remainder who live with disease maintain quality of life and function. However, chordoma is predominantly a surgically treated disease, and every step of care, from imaging, to biopsy, to surgery should be done by the ultimate treating expert. Treatment most typically involves a surgical resection with or without high-dose radiation therapy.
Dr. Matthew Colman at Northwestern Medicine in Chicago, Illinois in Chicago, Illinois is one of only a handful of experts in the country with the experience and skill set to manage this condition.
If located in the spine or sacrum, symptoms can include:
- Pain and aching at rest and independent of activity
- Numbness and weakness in the arms, legs, or perineum
- Bowel and bladder incontinence
- Often sacral chordomas do not cause symptoms until they grow very large, since they have space to grow around the tailbone and pelvis.
If located in the skull, symptoms can include:
- Headaches, neck pain
- Visual changes
- Difficulty swallowing
- Altered facial sensations or movement, voice, speech
Chordoma comes in several variants.
- The vast majority is called “conventional” chordoma and makes up 85% of cases. This variant is typically treated with surgery and/or radiation.
- Chondroid chordoma has a higher cartilage component and may have a better prognosis than the conventional variant.
- Dedifferentiated chordoma has a poor prognosis and is extremely high grade. However, it is commonly treated with and is known to respond to chemotherapy in addition to surgery.
There are no known risk factors. The majority occur as a random genetic event and not as the result of a heritable mutation.
Diagnosis involves a combination of imaging studies and biopsy. A plain x-ray will reveal the lesion. CT scan will provide better imaging detail of bony structures. An MRI is the best for delineating the extent of the chordoma in the soft tissues and for showing the nerves and spinal cord. A needle biopsy is necessary to confirm the diagnosis, but it is critical that biopsy and any other surgery is done at the treating center and by an expert in chordomas such as Dr. Matthew Colman. Typically the quality and appropriateness of the first treatment steps determines the ultimate success and outcome for the patient.
The main treatment is surgery, since the tumors are typically resistant to radiation and/or chemotherapy. Complete resection of the tumor in one piece with a wide margin of normal tissue (en bloc spondylectomy) offers the best chance for long-term control. However, the location may make it difficult to achieve complete removal without affecting critical structures.
Radiation therapy is used either before surgery, after surgery, or both in order to kill any cancer cells which are separate from the main mass or when surgery is not feasible. Advanced techniques like proton beam therapy or stereotactic radiosurgery are often employed due to their precision and ability to minimize damage to surrounding tissues.
While there are no specific drugs approved for chordomas, targeted therapies and chemotherapy may be considered in some cases, especially if the tumor is recurrent or metastatic.
Regular follow-up is essential to monitor for recurrence or progression of the disease. This typically involves periodic imaging studies and clinical evaluations. For the first 2-5 years after surgery, imaging will occur frequently to ensure no recurrence of the tumor.
Chordomas are rare, slow-growing tumors that can cause significant symptoms and complications due to their location along the spine and skull base. If you or a loved one experiences any of these symptoms, it is important to see an expert. Dr. Matthew Colman is an expert in bone tumors and chordomas. He can determine the cause of your symptoms and recommend appropriate treatment options.
Contact Dr. Matthew Colman at Northwestern Medicine in Chicago, Illinois. He specializes in degenerative spine conditions, spine trauma, spinal deformities and spine oncology. His patients find him to be a kind, caring, and thorough surgeon, who has a wonderful bedside manner, and is knowledgeable and trustworthy. They note that he takes time to listen to his patients, explains conditions well and answers their questions.
Dr. Colman’s care philosophy is that surgery should be a last resort. He feels strongly that conservative treatments including physical therapy and anti-inflammatory medications and injections should be pursed to their fullest extent before entertaining surgery. However, he knowns that certain orthopedic conditions require immediate surgical intervention for the best possible outcomes. Contact him at Northwestern Medicine in Chicago, Illinois to schedule a consultation to receive the correct diagnosis and all your treatment options today.
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Dr. Matthew Colman
- Internationally recognized expertise and thought leadership for a diverse range of spinal problems
- Patient-specific and humanistic approach which uses the latest technology and techniques
- Team centered approach prioritizes availability, communication, and support
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