What Is a Chordoma?
A chordoma is a particularly rare, malignant (cancerous) tumor that starts in base of the skull or the spine, most often in the bottom set of vertebrae called the sacrum. These bone sarcomas represent less than 4 percent of bone cancers and occur most often in people ages 40 to 70.
Chordomas appear to come from the notochord, a collection of cells that guides spine formation while a baby develops in the womb. Occasionally, pieces of the notochord stay in the body after the spinal bones fully develop. In some cases, those pieces don’t cause any problems and just need to be watched over time. In other cases, they turn into cancer, for reasons not yet fully understood.
It’s possible to develop more than one chordoma tumor at a time, in different places along the spine. These tumors are slow-growing but can still damage surrounding bone and invade nearby soft tissues. They also metastasize (spread) in 30 percent of cases, usually to the lungs.
The Sarcoma Program at Penn Medicine’s Abramson Cancer Center is home to one of the nation’s most experienced chordoma care teams.
Chordoma Symptoms
Most chordomas grow slowly, so they often don’t cause symptoms right away. If you do develop symptoms, they may vary depending on the tumor’s size and where it forms.
Symptoms from the skull base may include:
- Headaches
- Swallowing problems
- Vision changes
Symptoms from the mobile spine—the section that moves—may include:
- Pain near the tumor
- Symptoms from nerve compression—pain, weakness, numbness, tingling in arms or legs, or loss of coordination
Symptoms from the sacrum and the coccyx (tailbone)—the fixed, bottom parts of the spine—may include:
- Numbness in the groin
- Bowel and bladder changes
- Tingling, numbness, and weakness in the legs
What Causes Cancerous Chordomas?
In most cases, doctors don’t know why cancer develops from leftover notochord. Researchers continue to investigate this question, but finding answers is challenging because chordomas are so rare.
Unlike some other bone cancers, there is no evidence that chordomas have ties to previous medical treatments. However, some children who have a rare genetic condition called tuberous sclerosis develop chordomas, along with a range of other tumors. Sometimes tuberous sclerosis is inherited from a parent. At other times, the genetic changes responsible take place in the womb.
Chordoma Diagnosis
X-rays only provide a limited amount of information for chordomas, so we use additional imaging to provide more detailed images and confirm a diagnosis. These scans may include magnetic resonance imaging (MRI) and computed tomography (CT). It’s important to look from the base of the skull to the bottom of the spine, to check for the possibility of multiple tumors.
In addition to scans, we typically take a small sample of the tumor during a minimally invasive procedure called a biopsy. Analyzing the sample helps our team learn more about the cancer and plan treatment.
Learn more about sarcoma diagnosis and staging.
Chordoma Treatment at Penn
Because of the complex areas where chordomas form, they are often challenging to treat. Chordoma treatment often involves expert specialists from multiple disciplines to bring you the most comprehensive approach to sarcoma treatment. The precise partnerships depend on the tumor’s location but may include:
Removing chordomas with a minimally invasive operation provides the most effective treatment—as with sarcoma surgery for tumors in other locations, it’s best to remove these tumors in one piece. We may also take out some surrounding tissue to ensure all cancer comes out, carefully navigating around sensitive structures. Our team has expertise in this advanced approach. For skull base tumors, TransOral robotic surgery (TORS) may be an option.
Most chordomas are also treated with radiation therapy to reduce the risk that cancer returns. Depending on the circumstances, you may receive traditional radiation therapy, proton therapy, or a combination. Radiation therapy can also provide an option when surgery isn’t possible because of the tumor’s location near sensitive areas.
Even with the best treatment, chordomas can recur (return). To protect your health, we follow you closely after treatment ends. Regular checkups ensure that if cancer does return, we can treat it quickly. We may recommend additional options, including targeted therapy.
Make an Appointment
Please call 800-789-7366 or make an appointment.
The Penn Sarcoma Program offers same-day appointments to patients who have their imaging available. To learn more, call 215-349-8200.