Chordoma is a very rare type of cancer that occurs in the bones of the skull and spine in people of all ages. Chordoma tumor cells arise from cells of the notochord, a cartilage-like structure that acts like building blocks for the development of the spine during fetal development. Chordoma is part of a family of cancers called sarcoma, which includes cancers of the bones, cartilage, blood vessels, muscles, and other connective tissue. Please visit our Understanding Chordoma page for more information.

Chordoma is diagnosed in just one in one million people every year. At any given time, fewer than one in 100,000 people are living with chordoma.

Chordoma tumors develop from cells of a tissue called the notochordThe tissue in a fetus that acts as the building blocks for the spine. The notochord disappears when the fetus is about 8 weeks old, but some notochord cells are left behind in the bones of the skull and spine., which is a structure in an embryo that helps in the development of the spine. The notochord disappears when the fetus is about 8 weeks old, but some notochord cells remain behind in the bones of the spine and skull baseThe bones at the bottom part of the skull that separate the brain from other structures. The clivus is one of the bones of the skull base.. Very rarely, these cells turn into cancer called chordoma. What causes notochord cells to become cancerous in some people is still not fully known, but researchers are working to learn the answer and no specific environmental factors have been linked to chordoma.

A very, very small fraction of chordomas are hereditary (also called familial), which means they are caused by genetic mutations passed on to a person by their parents. For more information on familial chordoma, visit our Understanding Chordoma page.

The vast majority of chordomas occur at random and not as a direct result of an inherited genetic trait. There are only a handful of known cases where multiple members of the same family are affected by chordoma. This indicates that in these very rare instances, a strong genetic predisposition for chordoma can be inherited. If only one person in a family has been diagnosed, it is extremely unlikely that any other family members are at risk for developing chordoma. However, if more than one person in a family has been diagnosed with chordoma, other blood relatives may be at higher risk than the general population. More information about genetic risk factors for chordoma patients is available from the National Cancer Institute’s chordoma study and the ongoing Chordoma Genome Project.

A definitive diagnosis for chordoma can only be made by a pathologist who examines a sample of the tumor tissue under a microscope and tests for the presence of brachyuryA gene that makes a protein, also called brachyury, that is present at high levels in nearly all chordoma tumors.. You can learn more about the process for diagnosing chordoma in the video below.

For skull base tumors, biopsies can be risky so they are usually done at the time of surgery. For mobile spineThe parts of the spine not including the sacrum. The cervical spine (neck), thoracic spine (upper back), and lumbar spine (lower back) are the parts of the mobile spine. and sacral tumors, a trocar CT-guided biopsyA type of biopsy that uses a needle encased in a tube to retrieve a tumor sample and a CT scanner to guide the placement of the biopsy needle. This type of biopsy can help reduce the chance of spreading tumor cells. is recommended before any treatment is planned. You can learn more about biopsies for skull base and sacral chordomas in the video below.

Yes. Cancer is a term for a disease in which abnormal cells divide uncontrollably, invade other tissues, and can spread to other parts of the body. We now know that chordoma does invade other tissues and can spread, even though it is very slow-growing in most cases. For more information about the definition of cancer, see What is Cancer? by the National Cancer Institute.

Yes. Sarcomas are cancers of the connective tissue including bones, cartilage, and muscles. Chordomas arise from the bones of the skull base and spine.

Chordomas are not brain tumors. Chordomas of the skull base develop in a bone in the skull called the clivusThe surface of a portion of bone at the base of the skull. It is surrounded by the brainstem and both carotid arteries. Chordomas that form in this area are called clival chordomas., while brain tumors form from brain cells. However, skull base chordomas can affect the brainstemThe brainstem is the lower part of the brain connected to the spinal cord. The brainstem relays all signals to and from the brain and the body and is responsible for maintaining consciousness, breathing, and heartbeat. and can spread through the spinal fluid to the brain or other places on the spinal cord. Because of this, chordomas are sometimes considered brain tumors or central nervous system tumors, but they do not arise from brain cells as other brain tumors do.

Chordomas are considered malignant because they can invade other tissues, frequently recur (re-grow) after removal, and have the potential to spread to other parts of the body (metastasizeWhen tumors have spread to other parts of the body from the original tumor site.). Chordomas are considered a low-grade malignancyTumors that can invade and destroy nearby tissue and spread to other parts of the body, making them life-threatening. because they are relatively slow growing and they most often recur locally rather than spreading throughout the body. Because chordomas are low-grade, sometimes they are mistakenly called benign. Benign tumors do not invade other tissue, do not metastasize, and usually do not return after being removed.

Chordomas are most often described based on two things: their location in the body, and their appearance under a microscope. Chordomas occur in the locations of the skull base (head), cervical spineThe seven vertebrae that make up the neck. These vertebrae are commonly referred to as C1-C7. (neck), thoracic spineThe twelve vertebrae of the upper and mid back, extending from the shoulders to the bottom of the rib cage. These bones are commonly referred to as T1-T12. (mid-back), lumbar spineThe five vertebrae of the lower back, commonly referred to as L1-L5. (lower back), sacrumThe five vertebrae at the base of the spine near the pelvis, and between the two hip bones. These bones are commonly referred to as S1-S5. (pelvic area), and coccyxThe final segment of the human vertebral column, commonly called the tailbone. It consists of 3 to 5 fused vertebrae below the sacrum. (tail bone). Very rarely chordomas occur elsewhere in the body.

How they appear under a microscope determines the type of chordoma. Chordomas can be grouped into four different types: conventional (also called classic), poorly differentiated, dedifferentiated, and chondroid. See Understanding Chordoma for more information on these subtypes.

It is important to remember that the prognosisA term used for a doctor’s prediction of how a patient’s disease will progress. for each person is unique, and depends on many different factors. These include the patient’s age, type of chordoma, size and location of the tumor, method of treatment, extent of resection, and other factors. Only your doctors can advise about your individual prognosis and risks, and it’s very important that this advice come from doctors who have experience treating chordoma. With appropriate treatment, many chordoma patients will live for a decade or more, and some can be cured.

Surgery, radiation therapy, and systemic therapyThe use of drugs that spread through the body to kill cancer cells. Also called chemotherapy or drug therapy. are the main treatment approaches used to treat chordoma. How they are used and in what combinations depends on the patient’s case and stage of disease For chordoma, stage of disease refers to primary/new diagnosis, recurrent disease, advanced disease, and metastatic disease. This is different than other types of cancers that are usually staged by the numbers 0-4.. That is, whether chordoma has recently been diagnosed, has recurred after treatment, or has spread to other parts of the body. Visit the pages below to learn more.

• Surgery
• Radiation
• Systemic therapy
• Clinical trials
• Treatment guidelines for a new diagnosis
• Treatment guidelines for recurrence
• Advanced or metastatic disease

Every chordoma patient is unique, so different treatment approaches may be appropriate for different patients. There may even be several possible treatment options for an individual patient, each with different risks and benefits. It is important to carefully consider your options and get opinions from multiple doctors who have experience treating chordoma in order to make the most informed decisions. Read more about this on our Getting the Best Care page.

Appropriate diagnosis and treatment of chordoma requires very specialized care provided by multiple types of doctors. This team approach involving multiple specialists is called multidisciplinary care. Visit our Doctor Directory to find doctors who have experience treating chordoma and work in multidisciplinaryTreatment that involves a team of physicians from various disciplines. In the case of chordoma, these disciplines include sarcoma or bone pathology, radiology, spine surgery or skull base surgery, otolaryngology, radiation oncology, medical oncology, and palliative care. teams.

Experts recommend that your care team include specialists with experience diagnosing and treating chordoma in the following areas:

• Radiology
• Pathology
• Surgery
• Radiation oncology
• Medical oncology
• Palliative careCare given to improve the quality of life of patients who have a serious or life-threatening disease.

It is very important for chordoma patients to be treated by doctors who have substantial experience with chordoma. Read more about how to make this happen on our Getting the Best Care page.

You can learn more about what chordoma patients should look for in a treatment team in the video below.

It is the Chordoma Foundation’s policy not to recommend one doctor or hospital over another. Chordomas are complex and rare tumors, so it is important to find a doctor that has experience treating chordoma. Getting opinions from more than one experienced doctor will help you and your family make the most informed decision about your treatment. A list of doctors who have experience treating chordoma can be found in our Doctor Directory.

Doctors who have experience treating chordoma unfortunately cannot be found at every medical center, and many chordoma patients find they must travel to receive the best care. Our Doctor Directory can help you find experienced doctors, and as a first step you can use the information in the Directory to contact the doctors and request a remote consultation, so that you can get their initial opinions on treatment options over the phone or by email. If you find you must travel for treatment a CF Patient Navigator can help you learn about options for transportation and lodging assistance, if needed.

High doses of radiation (at least 74 GyE) are required to control chordoma tumors. To help protect vital anatomy such as the brain and spinal cord, radiation therapy for chordoma must be highly focused on the tumor while avoiding surrounding tissue. Particle therapy, such as proton beam therapy and carbon ion therapy, are most often recommended for chordoma treatment. Some types of conventional photon (x-ray) radiation can be used as an alternative, as long as they are very focused types that can deliver the recommended dose of radiation while minimizing the risk of damage to nearby organs and tissues. See the Radiation for Chordoma page of our website and the video below for information about different types of radiation used to treat chordoma.

The number of times you can have radiation depends on a number of factors. Every tissue in the body has a certain lifetime maximum tolerance for radiation, beyond which serious injury will occur. In some cases, delivering enough radiation to effectively treat chordoma means that vital surrounding structures (such as the brainstem or spinal cord) will be exposed to a dose of radiation close to the lifetime maximum. Delivering more radiation to those structures after initial treatment could be very dangerous.

If a tumor returns after initial radiation, the ability to have further radiation will depend on the location of the original tumor, the location of the recurrent tumor, the dose and distribution of radiation that was given initially, and other factors. Patients who experience a recurrenceTumor that has grown back after initial treatment. Recurrences can be isolated or multifocal, local or regional. after radiation should discuss treatment options with their radiation oncologist, and also seek at least one second opinion.

Our Clinical Trials Catalogue lists all chordoma-specific clinical trials that are open to chordoma patients, as well as a list of trials determined by our Medical Advisory Board to be relevant to chordoma. You can learn more on the Clinical Trials for Chordoma page of our website.

The Chordoma Foundation Medical Advisory Board recommends that chordoma patients with recurrent or advanced disease consider clinical trials in consultation with a medical oncologist who has experience with chordoma. Your doctor can help you consider any risks and potential benefits associated with particular clinical trials.

Clinical trials are important treatment options for any cancer patient to consider. Because standard therapies for chordoma are very limited, a clinical trial may provide an opportunity to receive a new, experimental therapy that may be more effective than the currently available options. It is important to remember, however, that the trial therapy may also be less effective than other options. Clinical trials may also make it possible for patients to gain access to clinically available therapies at a much lower cost than paying out-of-pocket for an off-labelThe practice of prescribing drug treatments that are not approved by government agencies to treat a particular disease. Doctors are allowed to prescribe drugs off-label if they believe it is in the best interest of the patient. therapy. In addition, chordoma patients who participate in clinical trials contribute to knowledge that can guide treatment for future patients and potentially help identify new ways of treating this rare cancer.

Learn more about participating in a clinical trial on this page of our website, as well as the videos below.

         

Because chordomas tend to be slow growing, they are generally resistant to conventional chemotherapy drugs which target and damage rapidly dividing cells. There is some evidence of poorly differentiatedA histological subtype of chordoma that is more aggressive and usually grows faster than the conventional type. It is more common in children and young adults, and is often characterized by loss of the INI1 protein. or dedifferentiatedA histological subtype of chordoma that is more aggressive and usually grows faster than conventional chordomas. Dedifferentiated chordomas occur in only 5 percent of patients, can have loss of the INI1 gene, and are more common in pediatric patients. chordomas showing response to chemotherapy drugs, but conventional chordomas typically do not respond.

If you or your loved one has a tumor that is determined to be poorly differentiated or dedifferentiated, the type of chemotherapy regimens that may be recommended for you include doxorubicin, ifosfamide, etoposide, vincristine, and cyclophosphamide.

Newer systemic therapies, such as targeted drug treatments and immunotherapies may hold promise for the treatment of chordoma. Currently, no drug therapies are approved by the Food and Drug Administration (FDA) or the European Medicines Agency (EMA) for use in chordoma patients. However, there have been a number of published reports of responses to various targeted drugs and medical oncologists can sometimes prescribe these drugs to patients “off-label.” Our Systemic Therapy Table includes a list of the types of drugs for which there is published evidence for use in chordoma. Learn more about systemic therapy for chordoma in the video below.

Every patient’s cancer cells have changes, or alterations, to certain genes. Molecular tumor profiling tests can be done on tumor samples to learn about the specific alterations found in the cells of an individual patient’s tumor, which can then help determine which targeted drug therapies might work best for a patient. This is a relatively new field of science and the tests do not always provide useful results. However, if they do provide useful results, medical oncologists can use these results to help guide their decisions about what drug to prescribe. This is sometimes referred to as personalized medicine, personalized oncology, or precision medicine. Your doctor may be able to perform the tests for you, and there are also a number of private companies that offer tumor profiling. Learn more about molecular tumor profiling on our Systemic Therapy for Chordoma page and in the video below.

There is no peer-reviewed evidence to support the use of alternative or complementary therapies for the treatment of chordoma. Alternative medicines are used in place of standard treatments, while complementary medicines are used together with traditional treatments. The field of integrative medicine brings together complementary and traditional practices to make sure they work well together, as some complementary therapies can have adverse interactions with drugs or treatments prescribed by your doctor. Integrative medicine departments can be found in many multidisciplinary medical centers.

Learn about the most important things for a chordoma patient to know in the video below.

The cost of treatment depends on many factors including the type of treatment you have, the length of your hospital stay, your rehabilitation/recovery needs, and your healthcare system or health insurance coverage. In the U.S., hospitals have worked out a set rate that insurance companies pay for treatments. If you are paying cash or are traveling to the U.S. for treatment that will not be covered by your own healthcare system, the hospitals set their own rates. Most medical centers will be able to give you an estimate of the cost of treatment when you are first consulting with doctors, and the center’s financial offices can discuss payment options with you as well.

Dealing with chordoma can pose a major financial burden. Government-sponsored programs, as well as services provided by other nonprofit organizations, may be available for people who do not have health insurance and for those who have insurance but still need financial assistance to cover healthcare costs. The Chordoma Foundation is a nonprofit organization and does not have the resources available to provide financial assistance for individual patients’ medical care; however, a list of organizations that provide financial assistance for cancer patients is available on the Helpful Resources page of our website.

Additionally, many medical centers have social workers or case managers who can assist patients with practical concerns, such as financial, travel, and lodging assistance programs. If the cost of treatment will be a difficulty, many medical centers have the option to create a monthly payment plan to help you pay for treatment over a period of time.

A Chordoma Foundation Patient Navigator can also help you find assistance programs to consider.

Support from others who have dealt with chordoma can be very helpful. The following resources may help you make connections to get that support.

The Chordoma Foundation Peer Connect Program is a free, confidential program that matches you with a trained Peer Guide from the chordoma community to help support you throughout your journey with chordoma. You can apply to be matched with a Peer Guide for support or to become a Peer Guide and provide support.

Chordoma Connections is our private, online patient community where individuals affected by chordoma can come together to exchange information, share experiences, and support one another. Click here to join Chordoma Connections and connect with others in the community.

Chordoma Survivors* is a private Facebook group where newly diagnosed patients, chordoma survivors, and family members can connect with others in the chordoma community. This group was created by and run by individuals who have personal connections to chordoma. This close-knit community exists to help answer questions, share personal experiences, and serve as encouragement throughout your journey with chordoma. Email approval from a group moderator is required to protect the privacy of the members. Please send an email briefly describing your relationship to chordoma to help@chordomasupport.org and you will receive a reply from a group administrator. Please note: While many members of this Facebook group are involved with the Foundation as volunteers and community advisors, the group is privately run and separate from the Foundation.

There is no specific diet or lifestyle that chordoma patients should adhere to. However, in general it is a good idea to eat a healthy diet and stay in good physical shape. This is especially true for cancer patients, as surgery, radiation, and drug therapyThe use of treatments like targeted therapies to kill cancer cells. can cause both emotional and physical stress. The healthier you are going into treatment, the greater your chances of a smooth recovery. Please consult with your doctor about any restrictions in your activities or diet that may be warranted.

Visit the Take Action section of our website to learn about the many ways to help advance our search for a cure. You can also contact our development team at development@chordoma.org to inquire about ways to get involved.

The Chordoma Foundation Tumor Donation Program collects tissue for our Biobank, a centralized repository of tumor tissue and blood contributed by chordoma patients to help advance research. To contribute, contact our Repository Manager, Patty Cogswell, at (877) 230-0164 or tumordonation@chordoma.org prior to your next surgery. We will make arrangements with the hospital to collect tissue remaining from your procedure that is not needed for your care. If you had surgery in the past, tissue from a previous surgery might be stored at the hospital where you were treated. Let us know when and where you had surgery, and we will attempt to locate and obtain this tissue. For more information, visit the Tumor Donation Program page.