Chordoma

For a list of resources where you can find out about chordoma research, visit the Research & Clinical Trials tab.

The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. Currently there are several studies enrolling people with chordoma and aim to improve treatment for this disease. After you click on a study, review its "eligibility" criteria to determine its appropriateness. Use the study's contact information to learn more. Check the ClinicalTrials.gov site often for updates.

You can also contact the Patient Recruitment and Public Liaison Office at the National Institutes of Health (NIH). We recommend calling the toll-free number listed below to speak with a specialist, who can help you determine if there are any other trials for chordoma.

Patient Recruitment and Public Liaison Office
NIH Clinical Center
National Institutes of Health
Bethesda, Maryland 20892,2655
Toll-free: 800-411-1222
Fax: 301-480-9793
Email: prpl@mail.cc.nih.gov

A chordoma is a rare tumor that develops from cells of the notochord, a structure that is present in the developing embryo and is important for the development of the spine. The notochord usually disappears before birth, though a few cells may remain embedded in the bones of the spine or at the base of the skull.

Chordomas typically present in adults between the ages of 40 and 70 and can occur anywhere along the spine. About half of all chordomas occur at the bottom of the spine (sacrum); about one third occur at the base of the skull. The remaining cases of chordomas form in the spine at the level of the neck, chest, or other parts of the lower back. Chordomas grow slowly, extending gradually into the surrounding bone and soft tissue. The actual symptoms depend on the location of the chordoma. A chordoma that occurs at the base of the spine may cause problems with bladder and bowel function. A chordoma at the base of the skull may lead to double vision and headaches.

In many cases, the cause of the chordoma remains unknown. Recent studies have shown that changes in the T gene _have been associated with chordomas in a small set of families. In these families an inherited duplication of the _T gene is associated with an increased risk of developing a chordoma. Duplications of the T gene have also been identified in people with chordoma who have no history of the tumor in their family, but in these cases the changes occur only in the tumor cells and are not inherited. The current treatment is often the surgical removal of the tumor, followed by radiotherapy.

The prognosis of chordomas generally depends on the success of the surgery removing the tumor. Although chordomas are usually slow-growing tumors, they are locally aggressive and tend to infiltrate into adjacent tissues and organs and to have multiple local recurrences (return of the tumor). Local recurrence results in tissue destruction and generally is the cause of death. Spreading to distant places of the body (metastases) are recognized but are uncommon. In general, a more complete removal with wide removal delays the time between surgery and eventual recurrence.

One studied showed that about 67% of the patients with chordomas of the base of the skull were alive after 5 years and in 58% of the patients the tumor did not get worse (progression-free survival) compared with the time of the diagnosis; about 57% of the patients were alive after 10 years and, in about 44%, the tumor did not get worse. The prognosis was better if more of the tumor was removed, the patient had radiation therapy, and there was no invasion of the nose and pharynx. A large study confirmed that both 5-year progression-free survival and overall survival of skull chordomas are better when there is a complete resection of the tumor.

Knowledge of the completeness of the tumor resection helps predict patient outcome in terms of the length of the time that the patient will not have any tumor recurrence, and in determination of the need for radiotherapy.

In general, the chordomas may recur after 3.8 years for radically resected tumors, 2.1 years for subtotal resection followed by radiation therapy, and 8 months for subtotal excision without radiation therapy. Due to the high rate of recurrences, frequent follow-ups are required because when the recurrence is identified early it is easier to treat.

Survival among pediatric patients who go through surgery was significantly longer than for adults and overall survival was longer.

Chordomas are treated with surgery because these tumors continuously grow, although they grow slowly. If the chordoma is not removed, it may wear away the bone and adjacent soft tissue, causing destruction of surrounding tissues. The surgery aims to remove as much of the tumor as possible. The extent of the surgery, or the amount of tumor that may be removed, depends on the location of the tumor and how close it is to critical structures in the brain. In some cases, surgery is followed by radiation therapy to destroy any cancer cells that may remain after surgery, especially when the tumor cannot be removed completely. Several studies have shown that carbon ion therapy or proton beam radiation may control tumor growth and improve survival.

Radical resections of tumors (removal of all the tumor) with clean margins (with no remaining of the tumor) are associated with a longer period of being disease-free. If the tumor cannot be removed completely, because of the location and closeness to critical delicate structures, the addition of radiation therapy decreases the recurrence of the tumor. Frequent follow-up is needed because of the high rate of recurrence of these tumors. Tumor recurrence identified early is easier to treat. The time in between follow-up visits, including repeat MRI or CT scans, depends on the completeness of the resection. Because residual tumor shortens the recurrence time, patients with known or suspected residual tumor need to be evaluated more frequently.