Chordoma Foundation


Frequently Asked Questions

About Chordoma

Treatment

Living with chordoma

Taking action


About Chordoma

What is chordoma?

Chordoma is a type of cancer that occurs in the bones of the head and spine in people of all ages. It arises from remnants of an embryonic structure called the notochord, which is the precursor to the spine. Chordoma is part of a family of cancers called sarcoma, which include cancers of the bones, cartilage, blood vessels muscles and other connective tissue. See Understanding Chordoma for more information.

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What causes chordoma?

All cancers are the result of mutations (changes) in a cell’s DNA, which cause the cell to acquire harmful properties such as the ability to divide infinitely, to invade other tissues, and to avoid destruction by the immune system.

Like most cancers, almost all chordomas are sporadic, meaning that they develop as the result of mutations that occur at random during the course of one’s life. While one’s environment contributes to sporadic mutations, there are no specific environmental factors that have been linked to chordoma.

A very small fraction of chordomas are hereditary (also called familial), meaning that they are caused by mutations passed on from one’s parents.  Some, but not all, cases of familial chordoma are caused by inheriting an extra copy of a gene called brachyury. There is also evidence suggesting that brachyury plays an important role in many sporadic chordomas as well, however more research is needed to determine if and how changes in the brachyury gene cause chordoma.

Identifying the genetic mutations that cause chordoma will greatly enhance understanding of the disease and could help lead to the development of more effective targeted treatments. The Chordoma Foundation is currently sponsoring the Chordoma Genome Project to catalog all of the genetic changes present in chordoma tumors in order to enable researchers to develop therapies that target these changes. Additionally, the National Cancer Institute is conducting a genetics study to identify additional hereditary causes of chordoma.

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Is chordoma a type of cancer?

Yes. Cancer is a term for a disease in which abnormal cells divide without control and can invade other tissues. For more information about the definition of cancer, see What is Cancer? by the National Cancer Institute

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Is chordoma a sarcoma?

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 cells are derived from a tissue called the notochord which is an embryonic cartilage-like structure. During development, notochord cells get lodged inside the bones of the skull and spine.

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Is chordoma a brain tumor?

Chordomas of the skull base often involve the brainstem and can spread through the spinal fluid to the brain or elsewhere on the spinal cord. As such, chordomas are sometimes considered brain tumors or central nervous system tumors, but they do not arise from brain cells.

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Are chordomas benign or malignant?

Chordomas are considered malignant because they can invade other tissues, frequently recur after removal, and have the potential to metastasize. Chordomas are considered a low grade malignancy because they are relatively slow growing and tend to recur locally rather than spreading throughout the body. 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.

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Are there different types of chordoma?

Chordomas are most often classified based on their location in the body and their histology (appearance under a microscope). Locations that chordomas can occur include the skull base (head), cervical spine (neck), thoracic spine (mid-back), lumbar spine (lower back), sacrum (pelvis area), and coccyx (tail bone). Extremely rarely chordomas occur elsewhere in the body.  Chordomas can be grouped into three different histologies: classic, chondroid, and dedifferentiated. See Understanding Chordoma for more information.

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What is the life expectancy for chordoma patients?

Each chordoma patient is unique. Age, gender, tumor location, size, histology, and underlying biology, as well as the nature of treatment received all factor into a patient’s outcome.

According to the latest and most comprehensive population-based study, the median survival for chordoma patients in the United States is approximately 7 years, and the overall 5, 10 and 20 year survival rates are 68%, 40% and 13%, respectively.

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Treatment

How are chordomas treated?

In most cases, surgery is needed to remove the tumor. The goal of surgery is to remove as much of the tumor as possible without causing unacceptable injury. Surgery is usually followed by radiation to control any part of the tumor that cannot be removed. Even if the entire tumor is removed, radiation is often given in order to kill microscopic tumor cells that could cause the tumor to return. In some situations, chordomas can be treated with radiation alone.

For recurrent chordoma, further surgery and/or radiation may be an option depending on where the tumor returns. It is common for chordoma patients to have multiple surgeries over the course of several years.

For advanced or metastatic chordoma that cannot be controlled with surgery or radiation, chemotherapy drugs may be used to slow the progression of the disease. There have been some reports of long-term responses to chemotherapy, however currently there are no drugs that are known to be curative. Clinical trials are ongoing to test the effectiveness of new targeted drugs for chordoma.

See the Treatment section of our website for more information about surgery, radiation, and chemotherapy options.

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What is the best treatment approach?

Each chordoma and each chordoma patient is unique; accordingly, different treatment approaches may be appropriate for different patients.  For a particular patient there might be several viable treatment  options, each with different risks and benefits, so it is important to carefully consider your options and get opinions from multiple different doctors with experience in treating chordoma.

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What types of doctors treat chordoma patients?

In general, chordomas are best managed by a multi-disciplinary team involving surgeons, radiation oncologists (who provide cancer treatment with radiation), medical oncologists (who provide cancer treatment with drugs), and other specialists. It is important that all members of your treatment team communicate and work closely together to coordinate your care.

Newly diagnosed patients should be evaluated by a surgeon with experience treating chordoma patients. Surgery for skull-base chordomas is should be performed by a neurosurgeon with specialized training in skull-base surgery, sometimes in partnership with an otolaryngologist (ears, nose, and throat surgeon). Chordomas of the spine and sacrum/coccyx should be treated by neurosurgeons or orthopedic surgeons with specialized training in spine surgery.

Depending on your particular case, your surgeon may refer you to a radiation oncologist for radiation treatment before and/or after surgery. Patients with recurrent, metastatic or inoperable disease, should be evaluated by a radiation oncologist and a medical oncologist.

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Which doctors should I go see/Which hospital should I go to?

It is the Foundation’s policy not to recommend one doctor or hospital over another. Given the complexity and rarity of chordoma, it is important to find a doctor that has experience treating chordoma and to get multiple opinions. Ultimately, you will have to make a decision based on what you and your family feel most comfortable with. A list of some of the doctors who we know have experience treating chordoma can be found in our Doctor Directory.

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What types of radiation therapy are available and how are they different?

Chordomas are radio-resistant meaning that very high doses of radiation are required to control these tumors. Because of their proximity to vital anatomy such as the brain and spinal cord, which cannot tolerate high doses of radiation, radiotherapy for chordoma must be highly conformal, meaning focused on the tumor while avoiding surrounding tissue. Several different types of conformal radiotherapy are available. See the Treatment section of our website for information about different types of radiation.

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Can I have radiation more than once?

Possibly, depending on a number of factors. It is important to realize that every tissue in the body has a certain life-time maximum tolerance for radiation, beyond which serious injury will occur. Sometimes, delivering enough radiation to effectively treat chordoma requires exposing vital surrounding structures (such as the brain stem or spinal cord) to a dose of radiation close to the life-time maximum. Delivering more radiation to those structures after initial treatment could be very dangerous.

If a tumor returns after initial radiation, it may or may not be possible to design a radiation treatment plan that is safe and effective. This 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 with a post-radiation recurrence should discuss treatment options with their radiation oncologist, and also seek at least one second opinion.

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What chemotherapy and drug treatment options are available?

Currently, there are no drug therapies that have been proven to be effective in a large portion of chordoma patients. Accordingly, the Food and Drug Administration (FDA) has not approved any drugs specifically for chordoma patients. However, that does not mean that there have not been good responses to particular drugs in particular chordoma patients, and, indeed, many chordoma patients benefit from chemotherapy. Medical oncologists are able to prescribe drugs for chordoma patients even if the FDA has not explicitly approved the drug for use in chordoma patients (this is called an “off-label” prescription).

Because chordomas tend to be slow growing, they are generally resistant to conventional chemotherapy drugs which target rapidly dividing cells. Newer, targeted drugs may hold more promise. There have been a number of published reports of responses to various targeted drugs. Click here for a list of all published responses to chemotherapy.

Increasingly, medical oncologists are attempting to customize therapies to the unique profile of each patient’s individual tumor (i.e. “personalized medicine”) in the hope that the treatments will be more effective and have fewer side effects. Your doctor may perform tests on your tumor to determine which targeted therapies make the most sense for you. A number of companies also offer tumor-profiling services that can help you make a rational decision about chemotherapy.

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What clinical trials are available for chordoma patients?

The Clinical Trials page lists all clinical trials that are known to be open to chordoma patients. Many of these trials do not explicitly list chordoma in the inclusion criteria, but each has been verified by a trial coordinator to be open to chordoma patients.

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Should I take part in a clinical trial?

There are benefits and risks of participating in clinical trials. Clinical trials can offer access to promising new treatments, often before they become available to the public. In addition to having the potential to benefit from the latest treatments, chordoma patients who participate in clinical trials will help researchers learn about chordoma and how to more effectively treat it. The more people who participate in clinical trials, the faster research questions can be answered that will lead to better treatment options for chordoma patients.

Before undergoing chemotherapy, it is worthwhile to discuss clinical trial options with your doctor. See the Clinical Trials page to learn more about clinical trials.

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Is there a role for complementary or alternative medicines to treat chordoma?

There is no peer-reviewed evidence that complementary or alternative medicines can improve outcomes for chordoma patients. Nonetheless, some patients may chose to include complementary or alternative medicines in their approach to fighting the disease. It is important to let your healthcare team know about all medicines or supplements that you are taking as some can have adverse interactions with drugs that your doctor prescribes.

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Living with chordoma

What do treatments for chordoma cost?

The cost for treatment varies by hospital.  If you have insurance, hospitals have worked out a set rate that the insurance company pays. If you are paying cash, they set their own rates. You will have to contact individual hospitals to find out what those rates are.

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Where can I find financial assistance?

Dealing with chordoma can pose a major financial burden. Government-sponsored programs as well as services provided by other nonprofit organizations are available for people who do not have health insurance and for those who have insurance but still need financial assistance to cover health care costs. An extensive list of financial assistance resources for cancer patients is available here.

The Chordoma Foundation’s mission is to help all chordoma patients by accelerating the development of effective treatments and ultimately a cure for chordoma. As such, we cannot provide financial assistance for individual medical care.

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How can I get support from others who have dealt with chordoma?

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Should family members or children of chordoma patients be concerned about getting chordoma?

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 highly unlikely that any other family members are at risk for developing a chordoma. However, if more than one person in a family has been diagnosed with chordoma, then other blood relatives are at risk for developing a chordoma.

Blood relatives of all chordoma patients should be vigilant, and should contact a doctor if they have any concern about the possibility of having a chordoma.

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.

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Does diet or lifestyle make a difference?

There is no specific diet or lifestyle that chordoma patients should adhere to. However, it is in general a good idea to eat a healthy diet and stay in good physical shape. That is especially true for cancer patients, as surgery, radiation and chemotherapy can be very taxing. The healthier you are going into treatment, the greater your chances are of recovering. Consult with your doctor about any restrictions in your activities or diet that may be warranted.

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Taking action

What can I do to help?

Visit the Take Action section of our website to learn about the many ways to help advance our search for a cure.

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How do I donate my tissue for research?

The Chordoma Foundation Biobank is a centralized repository of tumor tissue and blood contributed by chordoma patients to help advance research. To contribute, contact us at 877-230-0164 or biobank@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 biobank page.

 

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