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Chordomas develop in leftover cells from the notochord — a cartilage-like structure that helps form the early spine in the beginning stages of fetal development. Most of the notochord is replaced by the spine during the first six months of development. Small areas can remain, however, and it is believed that chordomas can form in these areas.

Chordomas can occur anywhere along the spine, but most often occur at the base of the spine (sacrum), the tailbone (coccyx), or the base of the skull.

Most chordomas occur in the sacrum, coccyx or base of the skull.

Most chordomas occur in patients between 40 and 70 years of age, with a slight predominance in males. Occasionally, they occur in younger patients—including children.

Chordomas are potentially life-threatening tumors. In some cases, they can spread, or metastasize, to other areas of the body. This can occur in up to 30% of patients. When chordomas spread, they most often spread to the lungs.

Chordomas occur spontaneously. They are not known to be caused by trauma, environmental factors, or diet. Chordomas are not inherited. They are not associated with any medical conditions or with the use of any medications or supplements.

Because chordomas are slow-growing, patients often have symptoms for a long time—in some cases, more than a year—before they seek medical attention.

Symptoms depend on the location of the tumor:

• Tumors in the skull can cause headaches, difficulty swallowing, or vision problems.
• Tumors in the spine can cause pain in the area of the tumor (neck, back, or tailbone). A tumor pressing on nearby nerves can cause symptoms similar to a slipped or herniated disk. These symptoms may include pain, weakness, numbness, clumsiness, or tingling in the arms or legs.
• Tumors in the tailbone area can cause a noticeable mass; numbness in the groin area; bowel and bladder problems; or numbness, tingling, and weakness in the legs.

Your doctor will perform a thorough physical examination and use imaging studies to diagnose a chordoma.

Imaging Tests

Several imaging studies may be needed to identify the tumor. These may include:

• X-rays
• Computed tomography (CT) scans
• Magnetic resonance imaging (MRI) scans

While chordomas themselves do not show up well on X-rays, an X-ray may reveal bone damage caused by the tumor. X-rays of the sacrum are often difficult for doctors to interpret.

An MRI is considered the best test for determining the total size of the tumor.

Cross-sectional CT scans of the pelvis (left) and the base of the spine (right) show a chordoma in the sacrum.

(Left) The same chordoma in the sacrum as it appears in an MRI scan taken from the side. (Center) How the chordoma appears in a cross-sectional MRI scan of the base of the spine and (right) of the entire pelvis.

Because of their proximity to the brain and spinal cord, chordomas are very difficult to treat.

Traditional chemotherapy is not usually very effective and standard radiation alone rarely cures the tumor.

Whenever possible, surgery is the preferred treatment option. Surgery is often combined with radiation to treat chordomas. If a tumor cannot be surgically removed, radiation therapy may help stop the growth of the tumor. Newer radiation techniques — such as proton beam therapy — may be more effective than standard radiation at stopping the tumor from growing.

Although it is the preferred treatment option, surgery can often be difficult to perform because of the important structures that are located near the tumor. To ensure the best outcome, the surgeon must remove the tumor and a portion of normal tissue surrounding it. This often results in some loss of neurologic function. For example, removing tumors in the sacrum may cause:

• Loss of bowel and bladder control (incontinence)
• Lower extremity weakness
• Loss of sensation in the groin area
• Instability of the pelvic bones.

Your doctor will talk with you about the risks in your specific case.

Your outcome after treatment depends on a number of factors, including:

• Your age
• The size and location of the tumor
• The method of treatment

Your surgeon will talk with you about your individual prognosis.

Advances in radiation therapy have allowed patients to receive higher doses of radiation with less damage to surrounding normal tissues, such as the brain and spinal cord. Different types of radiation therapy (including proton beam therapy, hadron therapy, intraoperative radiation, and brachytherapy) may offer better treatment of chordomas. These radiation therapies may be combined with more limited surgery in the future.

New drug treatments, including the use of a drug called imatinib mesylate, are currently being investigated. These drugs may help slow or stop the growth of tumors that cannot be removed with surgery.