Chordoma is a rare malignant bone tumor. A group of cells known as the notochord come together during the fourth through six weeks of fetal development. The notochord serves as the base for the development of the spinal cord. As the spinal cord forms, the notochord disappears, except for parts that form part of the discs between the vertebrae. Chordomas form from parts of the notochord that don’t break down as they should. Over time these leftover cells can change and become malignant. Harris Center researchers are studying these changes to develop new therapies for chordoma. Chordoma primarily spreads by local invasion, but it does have the potential to spread to the lungs, bone and liver.

Most develop at the base of the skull, the base of the spine or tailbone, but they can occur in other areas of the spine. Although they may develop at any age, tumors at the base of the skull most commonly appear between the ages of 35-40. Those that arise from the sacrum usually appear between the ages of 50 and 70. Approximately 300 new cases of chordoma are diagnosed in the US each year. Chordoma occurs more frequently in men. There are no known risk factors for chordoma.

What are the Symptoms of Chordoma?

 How is Chordoma Diagnosed?

Abnormalities are usually evident during a neurological examination, but imaging studies are needed. Magnetic Resonance Imaging (MRI)   is the most useful test. In addition, Computer Tomography (CT) study may be done to rule out spread of chordoma to other areas of the body. To make the final diagnosis, a biopsy is usually required.

Because Chordoma develops close to vital structures in the central nervous system, treatment is complex and can be difficult, but the prognosis is not always grim. In a recent study, Harris Center clinicians achieved a five-year continuous disease-free survival rate of 62.5% with primary sacral chordoma.

Click here for the PubMed summary of a recent paper by Harris Center clinicians on the efficacy of chordoma surgery performed in concert with proton-beam therapy.

Chordoma Surgery

The primary treatment for chordoma is the complete surgical removal of the tumor. To ensure the best outcome and prevent its spread, the surgeon must remove the tumor and a margin of normal tissue around it. The results of the first surgery largely determine the patient’s prognosis and it is important that an experienced team perform the procedure.

Radiation Therapy for Chordoma

Pre and/or postoperative radiation therapy has improved local control of chordoma. It can also slow the growth of the tumor if it has been incompletely resected or if it has recurred.

Proton beam therapy uses energy from protons of atoms to destroy cancer cells. It can be aimed at a tumor very precisely and can be concentrated on the tumor with little harm to the surrounding tissue.

For some patients for whom surgery is not a good option, high-dose radiation with a combination of x-rays and protons has been able to control tumors for over a decade and may be an important option for them.

Click here for information on proton beam therapy at Mass General Hospital

Chemotherapy

Chemotherapy has not proved effective in the past, but there are a number of clinical trials with new agents currently underway.