Although first described in 1856, the cause of chordoma is still unknown. In most cases chordomas are sporadic (occur randomly), however several families have been identified with multiple affected family members. This implies that inherited mutations (changes) in a gene or genes can cause some chordomas. Somatic (non-inherited) mutations in that same gene may be responsible for some or all sporadic chordomas. Identifying the gene or genes that cause chordoma will greatly enhance understanding of the disease and could help lead to the development of targeted treatments.
Chordomas are thought to arise from remnants of an embryonic rod-shaped structure similar to cartilage called the notochord. During early embryonic development the notochord provides structural rigidity to the embryo and serves as a scaffold for the formation of the spinal column and skull. As the bones of the spine and skull form, the notochord gradually disintegrates, and by birth only remains as scattered cells in the center of the intervertebral discs (IVD). In humans notochordal cells in the IVD become increasingly sparse after birth and disappear all together by adolescence.[1] One in five people, however, have small remnants of notochord in their vertebrae (not IVD) and/or skull base that can persist throughout life.[2] It is believed that these intraosseous (inside-the-bone) notochordal remnants, sometimes called benign notochordal cell tumors (BNCT), undergo a malignant transformation which causes uncontrolled proliferation (growth of cells), and ultimately leads to the formation of a chordoma.
Chordomas tend to return and spread in the areas adjacent to the original tumor location. Infrequently chordomas metastasize to distant parts of the body including the lungs, liver, and skin.
Yes. Chordomas have all the characteristics of a malignant tumor, with local invasiveness (growth in to nearby tissues), tendency for recurrence, and potential to metastasize. Chordomas are considered a low grade malignancy because they are relatively slow growing and tend to recur locally rather than spread systemically. Because chordomas are low grade, they sometimes are mistakenly called benign. Benign tumors do not invade other tissue, do not metastasize, and usually do not return after being removed.
Yes, chordoma is a type of cancer. Cancer is a term for diseases in which abnormal cells divide without control. More specifically, chordoma is a type of sarcoma - a term used to describe cancers of the connective tissue and bone.
The overwhelming majority of chordomas occur sporadically (at random), however a small number of families have an inherited genetic predisposition to develop chordomas. If only one person in a family has been diagnosed it is unlikely that any other family members would be at risk for developing a chordoma. However, if more than one person in a family has been diagnosed with chordoma then there is a high liklihood that other blood relatives would also be at risk for developing a chordoma.
Blood relatives of all chordoma patients should be vigilent about symptoms indicitive of a chordoma, and should contact a qualified medical professional if they have any concern about the possibility of having a chordoma.
For more information about genetic risk factors for chordoma check out the National Cancer Institute's
Familial Chordoma Study.
Currently, surgery and radiation are the only standard treatment options for chordoma. For recurrent, advanced, or metastatic chordoma, chemotherapy can be used to stop or slow the spread of disease. There have been few published reports of chemotherapy having substantial clinical benefit, however new targeted anti-cancer drugs may hold some promise. Clinical trials can offer access to the newest and most advanced anti-cancer drugs, even before they reach the market.
Surgery for skull base chordomas is usually performed by neurosurgeon and/or an otolaryngologist (ears, nose, and throat surgeon), depending on the surgical approach. Chordomas of the spine and sacrum/coccyx are treated by neurosurgeons or orthopedic surgeons specializing in spine surgery. Because maximal resection is essential for controlling the tumor, it is important to choose a surgeon who specializes in treating tumors of the skull base or spine.
To ensure that treatment is planned in the most rational fashion chordomas should be managed in a collaborative manner by a team of doctors including surgical oncologists (who provide cancer treatment with surgery), radiation oncologists (who provide cancer treatment with radiation), and medical oncologists (who provide cancer treatment with drugs). Many medical centers hold regular tumor boards in which a number of doctors who are experts in different specialties review and discuss the diagnosis and treatment options for each patient. Talk with your doctor about having your case reviewed by a tumor board.
See our
doctor database for a list of patient-recommended specialists.
The Chordoma Foundation is leading a coordinated multidisciplinary research effort to develop effective treatments and ultimately a cure for chordoma. By breaking down barriers to research we enable scientists to accelerate their search for a cure. Right now, the two biggest barriers are scarce funding and insufficient chordoma tissue available for research.
Save your tumor!
Fresh tumor is the starting point for virtually all research on chordoma. Surprisingly, most hospitals do not routinely save chordoma tissue (beyond what is needed for pathologic diagnosis), meaning that this valuable resource is lost forever. Your tissue is your property and it is literally worth its weight in gold. If you have not yet had surgery, you can help advance chordoma research by requesting that your tumor tissue be frozen. Before your surgery, you should tell your surgeon verbally and/or in writing that you want as much of your tumor saved as possible. After surgery you should request confirmation from your surgeon and/or pathologist that your tumor was in fact frozen.
In the near future, the Chordoma Foundation will set up a centralized BioBank (a specialized facility where tissue is stored) that will serve as a resource to the scientific community. At that time, you will be able to request that a portion of your tumor be sent directly to the Chordoma Foundation. For now, please notify us when you have a surgery by e-mailing with your name, your surgeon's name and hospital, and date of surgery. Once the BioBank has been set up we will follow up with you and your surgeon to retrieve the tissue.
Donate!
In order to establish the BioBank, procure tumor tissue and implement our research action plan, we need to raise $3 million by the end of 2009. You can help by making a donation and encouraging your friends and family to donate as well. There are no other organizations dedicated to curing chordoma so donating to the Chordoma Foundation is the most direct way to help bring about a cure. Your donation is an investment in the lives of all chordoma patients. We depend on your support and appreciate any contribution that you can make.
Ask your doctor for a referral to a professional counselor familiar with oncology care.
The
Chordoma Support Group is a community of over 400 active members from around the world. The members and managers of the group offer a wealth of experience, support and compassion.
Friends Health Connection offers a worldwide support network that links individuals with the same health problems on a customized, one-to-one basis for mutual support. Please tell them that you were referred by the Chordoma Foundation.
Only your doctor(s) can give you medical advice. Information on this website and the chordoma support group is strictly for educational purposes and should not be considered medical advice.
Oklahoma City
University of Pennsylvania
References »
[1] A. Peacock, Observations on the postnatal structure of the intervertebral disc in man. J Anat 86 (1952) 162-79.
[2] T. Yamaguchi, S. Suzuki, H. Ishiiwa, and Y. Ueda, Intraosseous benign notochordal cell tumours: overlooked precursors of classic chordomas? Histopathology 44 (2004) 597-602.