Chordoma is a rare cancerous (malignant) primary bone tumor that occurs along the spine, most often in the base of the spine (sacrum) or in the base of the skull. Chordomas can occur at any age, but they usually occur in adults. Although slow growing, chordomas are difficult tumors to treat, and they are often life-threatening.

Chordomas form from small remnants of the notochord, a coil of cells in the embryo that develops into the spinal cord. In rare cases, a leftover cell (notochord remnant) can develop into a chordoma. Symptoms depend on the site of the tumor. Long-term follow-up care is necessary after treatment ends because chordomas can spread (metastasize) and recur.

  • Expertise and experience. Because chordomas are rare, it can be difficult to find a medical center experienced in their treatment. Mayo Clinic's experienced specialists treat more people with chordomas than do most other centers in North America.
  • Multispecialty team. Depending on your needs, your Mayo Clinic team may include doctors from a number of specialties, such as orthopedic surgery, oncology, radiation oncology, neurosurgery and others. The rehabilitation team helps people recover function and independence after chordoma surgery.
  • Advanced technology. Mayo Clinic surgeons are leaders in the use of computer-assisted surgery to protect the critical structures of the brain and spine. Mayo's experts work with state-of-the-art imaging equipment.
  • Comprehensive cancer center. Mayo Clinic Cancer Center meets strict standards for a National Cancer Institute comprehensive cancer center, recognizing scientific excellence and a multispecialty approach focused on cancer prevention, diagnosis and treatment.

Diagnosis

Your doctor will perform a comprehensive physical exam and order tests to determine the stage and severity of your condition. Tests may include those below.

Biopsy

Image-guided needle biopsy is necessary to confirm a chordoma diagnosis. In this procedure, the doctor performs a needle biopsy during imaging that helps guide the needle precisely into the tumor. During the biopsy, the doctor removes cells from the abnormal area for examination under a microscope by Mayo Clinic's experienced pathology staff.

Imaging studies

You may have imaging tests, such as an MRI or a computerized tomography (CT) scan, to help locate changes in your brain or spine and to determine whether the cancer has spread. In tests that involve radiation, specialists carefully monitor doses to avoid the risk of radiation overexposure.

Read more about MRI and CT scan.

Treatment

A team of specialists in orthopedic surgery, medical oncology, radiation oncology, neurosurgery, and others as needed, treat chordoma using one or more of the following options:

  • Surgery. The primary treatment for chordoma is surgery, although it may be difficult to perform because the tumor is near critical structures in the brain and spinal cord. The goal of surgery is to remove the entire tumor in one piece, whenever possible, and Mayo surgeons have the expertise and experience to increase the chances of successful surgery.
  • Radiation therapy. Radiation therapy is used in combination with surgery to help stop tumor growth and recurrence. Radiation oncologists apply high-energy external beam radiation to tissues containing cancer cells. Radiation therapy may be used as a supplement before or after surgery or for cases when the tumor cannot be safely removed.
  • Chemotherapy. Chordomas are not generally treated with chemotherapy, although chemotherapy is sometimes effective for certain cases in killing rapidly multiplying cells in certain types of tumors.
  • Targeted therapy. Targeted therapies are drugs that aim for specific cancer-causing molecules. In unique circumstances, targeted therapies may be used for advanced chordomas.

Mayo Clinic in Rochester, Minn., is ranked among the Best Hospitals for cancer by U.S. News & World Report. Mayo Clinic in Jacksonville, Fla., and Mayo Clinic in Scottsdale, Ariz., are ranked high performing for cancer by U.S. News & World Report.

At Mayo Clinic, we assemble a team of specialists who take the time to listen and thoroughly understand your health issues and concerns. We tailor the care you receive to your personal health care needs. You can trust our specialists to collaborate and offer you the best possible outcomes, safety and service.

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Our patients tell us that the quality of their interactions, our attention to detail and the efficiency of their visits mean health care — and trusted answers — like they've never experienced.

Why Choose Mayo Clinic

What Sets Mayo Clinic Apart

Mayo Clinic works with hundreds of insurance companies and is an in-network provider for millions of people. In most cases, Mayo Clinic doesn't require a physician referral. Some insurers require referrals or may have additional requirements for certain medical care. All appointments are prioritized on the basis of medical need.

At Mayo Clinic in Arizona, chordomas are treated by experienced specialists in multiple areas, including medical oncology, neurological surgery, orthopedic surgery, radiation oncology, and others as needed.

For appointments or more information, call the Central Appointment Office at 800-446-2279 (toll-free) 8 a.m. to 5 p.m. Mountain Standard Time, Monday through Friday or complete an online appointment request form.

At Mayo Clinic in Florida, chordomas are treated by experienced specialists in multiple areas, including oncology, neurosurgery, orthopedic surgery, radiation oncology, and others as needed.

For appointments or more information, call the Central Appointment Office at 904-953-0853 8 a.m. to 5 p.m. Eastern time, Monday through Friday or complete an online appointment request form.

At Mayo Clinic in Minnesota, chordomas are treated by experienced specialists in multiple areas, including oncology, neurosurgery, orthopedic oncology, radiation oncology, and others as needed. The primary site for pediatric cancer care is the T. Denny Sanford Center for Pediatrics, although chordomas in children are very rare.

For appointments or more information, call the Central Appointment Office at 507-538-3270 7 a.m. to 6 p.m. Central time, Monday through Friday or complete an online appointment request form.

See information on patient services at the three Mayo Clinic locations, including transportation options and lodging.

Mayo Clinic Cancer Center meets strict standards for a National Cancer Institute comprehensive cancer center, which recognizes scientific excellence and a multispecialty approach focused on cancer prevention, diagnosis and treatment.

Publications

See a list of publications by Mayo Clinic doctors on chordoma on PubMed, a service of the National Library of Medicine.

Nov. 16, 2012