Chordomas are a type of primary bone cancer usually found in the skull or spine. These slow-growing tumors are rare, accounting for only one percent of cancers of the central nervous system. Chordomas develop from remnants of notochord, which precedes the spinal column during embryonic development. While chordomas may be found at any site along the axial skeleton, they are most frequently found at the sacrum (base of the spine), the coccyx (tailbone), and at the clivus (base of the skull).
Chordomas rarely metastasize, but are aggressive locally and affect adjacent organs, tissues, and bone. Symptoms can include back pain, numbness, headaches, and vision problems. This type of tumor has a high recurrence rate. Chordomas are most commonly found in adults between the ages of 40 and 70.
The preferred surgical treatment at UPMC for chordomas of the skull base is the Endoscopic Endonasal Approach (EEA) to remove the tumor. This innovative, minimally invasive technique uses the nose and nasal cavities as natural corridors to access hard-to-reach or previously inoperable tumors. EEA offers the benefits of no incisions to heal, no disfigurement to the patient, and a faster recovery time. If complementary treatments such as radiation therapy or chemotherapy are needed, those therapies can begin soon after surgery.
Your physician will perform a physical exam and will ask about your symptoms.
Skull base chordoma symptoms may include:
- headache
- double vision
- facial pain
- changes in hearing
- difficulty swallowing
In addition to a physical exam, your physician may request imaging tests, such as MRI or CT scans. Early diagnosis may prevent serious complications.
Three standard treatments are used: surgery, chemotherapy, and radiation therapy. Your physician may recommend one or a combination of these treatments. Frequent follow up is required because of the high recurrence rate of these tumors.
Surgery
Chordoma surgery is the main type of treatment for bone cancer. The procedure depends on the size and location of the tumor.
Chordomas of the skull base may be approached directly by using the Endoscopic Endonasal Approach (EEA). This approach allows surgeons to see the tumor well without making incisions to the face or skull. The tumor is removed through the nose and nasal cavities, and recovery time is faster than with the traditional approach. Patients can receive radiation treatment or chemotherapy sooner when indicated.
Radiation
Radiation therapy may be delivered externally by directing radiation at the tumor from an outside source, internally by placing radioactive material directly in the body near the cancer, or by using stereotactic radiosurgery to deliver a concentrated dose of radiation directly to the brain tumor. Proton beam radiation is the preferred type of radiation treatment for chordomas.
Chemotherapy
Chemotherapy uses drugs to stop the growth of cancer cells. Depending on the type and stage of the cancer, chemotherapy may be taken by mouth, as an injection, or placed directly into the tumor site. Modern chemotherapy for chordomas is under investigation at UPMC.
