Chordoma is a rare type of cancer that occurs in the bones of the skull base and spine. It is part of a group of malignant bone and soft tissue tumors called sarcomas. Chordomas account for about three percent of all bone tumors and about 20 percent of primary spinal tumors. They are the most common tumor of the sacrum and cervical spine. A chordoma tumor usually grows slowly, often without symptoms at first, and then might cause symptoms for years before doctors find it.

Chordomas are complicated tumors to treat due to the involvement of critical structures such as the brainstem, spinal cord, and important nerves and arteries. They can also come back, or recur, after treatment — usually in the same place as the first tumor. This is called a local recurrence. In about 30 to 40 percent of patients, the tumor eventually spreads, or metastasizes, to other parts of the body.

Chordomas are usually located in the clivus (the base of the skull) and sacrum (pelvis). They are also seen throughout the mobile spine in the cervical, thoracic and lumbar regions.

Chordomas are best seen on MRI scans, in particular MRI T2 weighted images. They tend not to enhance, but sometimes can enhance with IV contrast (Gadolinium). Chordomas do not consistently show up on PET CT or bone scans.

Because we treat chordomas aggressively, establishing the correct diagnosis before embarking on a treatment plan is critical. MRI scans cannot provide a definitive diagnosis; that’s why we recommend a CT-guided biopsy prior to treating any suspected spinal or sacral chordoma. (Chordomas in the clivus are not usually biopsied beforehand unless radiation is the only planned treatment.)

A biopsy can be percutaneous (a needle through the skin) or open (through surgery). To reduce the risk of spreading tumor, spinal and sacral chordomas should be biopsied using CT imaging and a needle through the skin. The biopsy should be planned so that the tissue along the path the needle takes to the tumor can then be removed with the tumor, if possible. Most biopsy needles today have a dual needle design in which one needle sits inside the other; this decreases the chance of tumor cells spreading to the biopsy tract.

The best treatment for a chordoma is to remove it in one piece (‘en bloc resection’) with negative margins. That means it should be completely cut out, leaving only healthy tissue. Patients undergoing this treatment have the best chance afterwards to live disease free, or even be cured.

One or more surgeries, called stages, may be needed. The surgery plan will be based on the location, shape and size of the tumor. Nerves and arteries may be affected by the tumor or treatment. Our physicians’ expertise with chordomas means they can provide extensive information on how this might affect you and your family.

In some cases, if your physician is concerned that a negative margin might be difficult to achieve, pre-operative radiation therapy will be considered. This therapy can be delivered with proton beam or stereotactic radiosurgery.

Removal of a chordoma in the spine is called a spondylectomy (or multi-level spondylectomy). In the sacrum, it’s referred to as a sacrectomy or sacral amputation. Because these surgeries are so extensive, surgeons from multiple specialties work together to achieve success. It’s critical that surgeons treating these tumors have extensive knowledge and experience performing en bloc resections.

All tumors are sensitive to radiation therapy, but different tumors require different doses of radiation to be destroyed. Sophisticated radiation techniques, such as proton beam therapy, can deliver high doses of radiation to a tumor. Chordomas, however, need a significantly higher dose of radiation therapy to be destroyed — a dose too toxic for the body to endure.

Carbon-ion radiation has recently been developed for treating tumors. Carbon-ion facilities currently are only in Japan, Italy and Germany. Researchers at these facilities hope to learn whether carbon-ion radiation may be effective on chordomas.

Chemotherapy is also not an effective first-line treatment for chordomas. Patients with reoccurring tumors may, however, be candidates for clinical trials. Your physician will look at the genetic makeup of your tumor and weigh whether personalized medicine involving chemotherapy would be beneficial.

Treating a chordoma requires a complex, well-managed care plan. It is critical that patients with chordomas get their treatment at centers with specialists who are experienced in treating these tumors. It’s not uncommon for patients to travel across the country or internationally to be treated by centers equipped to deliver such care.

Most patients stay one or two nights in the neurosurgery intensive care unit after their tumor has been removed. This time is usually uneventful for a patient, despite close monitoring by their care team.

Patients are then transferred to regular care on the neurosurgery unit, where they may stay for up to two weeks. During this time, physicians work to control post-operative pain, and physical and occupational therapy begin. Once the hospital stay is complete, some patients are ready to go home; others may need rehabilitation at an inpatient facility.

Northwestern Medicine patients needing additional rehabilitation work frequently with Northwestern Medicine Marianjoy Rehabilitation Hospital in Wheaton, Illinois or the Shirley Ryan AbilityLab (formerly the Rehabilitation Institute of Chicago).

Cervical nerves

There are seven bones of the neck and eight nerves; there are two nerve roots, one on either side, that exit at each level of the spine. The bones and nerves share the same medical terminology: C1, C2, C3, C4, C5, C6, C7 (and C8 for the eighth neck nerve).

• C1 – Loss of this nerve is not noticeable.
• C2 – Regulates sensation up the back of the head. Losing this nerve results in numbness and tingling up the back of the head on the side that the nerve is cut.
• C3, C4, C5 – These nerves form the phrenic nerve that moves the diaphragm up and down for breathing. Cutting C3 does not usually result in a significant change; cutting more than one nerve can decrease the ability of the diaphragm to move and may result in impaired breathing.
• C5 – Important for moving your arm above your shoulder. Loss of this nerve results in weakness of the shoulder on the side that the nerve is cut; patients cannot elevate the arm above chest height.
• C6 – Important for biceps function; the bending movement of the arm comes from the biceps. Weakness of C6 can negatively affect this muscle. The function of the biceps comes from C5 and C6.
• C7 – Important for triceps function. Straightening the arm comes from the triceps. Patients may be less impacted by the loss of this function in daily activities because gravity can be used to pull the arm down. People who rely more heavily on their triceps (for activities such as push-ups) will notice if this nerve is lost.
• C8 and T1 – Critical for using one’s hand. Weakness of these nerves results in loss of hand function, including bending and straightening of the fingers and the wrist.

Thoracic nerves

Most people have 12 bones of the spine, T1 through T12. The accompanying nerve at each level shares the same name.

These nerves mostly provide sensation around the chest on one side, starting at the spine and around to the front breast bone (sternum) and belly. Losing one of these nerves results in numbness, although most people find they are not bothered by this loss.

Lumbar nerves

There are five bones of the lumbar spine, L1 through L5, and five nerves of the lumbar spine, L1 through L5.

• L1, L2 – Important for the iliopsoas muscle, which bends the leg at the hip in order to lift one’s leg. Loss of L1 is usually not that significant.
• L2, L3, L4 – Form the femoral nerve that controls the muscles on the front of the leg, such as your quadriceps. The L4 nerve is the largest, most important nerve of the three. Loss of L4 results in leg weakness. The loss of L2 or L3 may not be as significant to some patients, but loss of the entire femoral nerve results in weakness in all front leg muscles. People can walk without this function, but they may have an unstable knee and need a knee brace. Most patients with this weakness must ‘throw’ the leg forward to walk.
• L5 – This nerve supplies the nerves to the muscles that raise the foot and big toe.

Sacral nerves

The sacral plexus is a nerve plexus which provides motor and sensory nerves for the posterior thigh, most of the lower leg and foot, and part of the pelvis. It is part of the lumbosacral plexus and emerges from the lumbar vertebrae and sacral vertebrae (L4-S4).

• S1 – Important for downward pressure of the foot. During a total sacrectomy, these nerves are sacrificed. Many patients do not notice this weakness during routine activities.
• S2, S3, S4 – Important for bowel, bladder and sexual function. If the S4 nerve is not preserved, most patients have near-normal bowel, bladder and sexual function. If the S2, S3 and S4 nerves are preserved on only one side, most patients have near-normal bowel, bladder and sexual function. If the S2 nerves are intact, but both S3 and S4 nerves are not, most patients will have some bowel, bladder and sexual function, but it will not be normal. If none of the S2, S3 and S4 nerves are preserved, all bowel, bladder and sexual function is lost. By following a specialized diet and using nighttime suppositories, these patients can adapt over time. A strict bladder catheterization program (catheterizing at least four times per day) allows most patients to avoid overflow incontinence during the day.

Yes. Some chordomas can reoccur, even after the entire tumor and its surrounding tissue have been removed. Treatment options may then involve removing the new tumor, radiation therapy or chemotherapy. Although radiation and chemotherapy have not been proven effective in destroying chordomas, these options may be tried to prevent growth and spread of the tumor. Patients with a reoccurring tumor may also consult with their care team as to whether a clinical trial is an option.

Since these tumors can reoccur — sometimes years later — we recommend lifelong monitoring for patients. Monitoring plans are tailored specifically for each patient; often this involves an MRI evaluation every 6 months.

Many patients who have had chordomas removed from their spine or sacrum will have their spine stabilized and fused. During this surgery, bone tissue is grafted; as it grows (fuses), it lessens the body’s reliance on the screws, rods and other instrumentation your physician may have used to stabilize your spine. Physicians usually monitor this process with X-rays.