Whether you’re newly diagnosed, experiencing a recurrence, or coping with advanced disease, it’s important to understand your options, seek care, and find support. Remember, you are not alone. The Chordoma Foundation and a community of other individuals affected by this disease are here to support you throughout your journey, from diagnosis to survivorship.

About chordoma

Chordoma is a rare cancer that occurs in the bones of the skull base and spine, affecting about 1 in 1 million people annually. That means that about 600 patients are diagnosed with chordoma each year in Europe. It grows slowly, often without symptoms at first.

Chordomas are difficult tumors to treat because they often involve important structures like the brainstem, spinal cord, and major nerves and arteries. In addition, they tend to return or recur after treatment, typically at the original tumor site, which is called a local recurrence. In about 30 to 40 percent of cases, the tumor may spread, or metastasize, to other parts of the body.

There are three types of chordoma. Conventional chordoma is the most common type, typically slow-growing and composed of unique cells with a bubbly appearance. It expresses the brachyury protein, which is a hallmark of chordoma and helps distinguish it from other tumors such as chondrosarcoma.

Dedifferentiated chordoma is a very rare subtype, occurring in 5 percent or less of patients. It tends to be more aggressive, grows faster, and is more likely to metastasize than conventional chordoma. Treatment options may differ from those for conventional chordoma, including chemotherapy. This subtype appears as a mixture of conventional chordoma cells that express brachyury and cells resembling high-grade sarcoma that do not express brachyury.

Poorly differentiated chordoma is another very rare subtype, officially recognized for the first time in the 2020 edition of the WHO classifications. It is typically more aggressive, faster growing, and more likely to metastasize than conventional chordoma. This subtype is more common in younger patients and those with skull base and cervical tumors. It may require different treatment options, including chemotherapy, and is defined by the deletion of a gene called SMARCB1 or INI1.

Treatment

The Chordoma Global Consensus Group (CGCG) — a multidisciplinary, international group of over 60 doctors who have extensive experience caring for chordoma patients — has developed guidelines for the diagnosis and treatment of chordoma.

You can find the guidelines here on our US website:

  • Guidelines for skull base tumors: The goal of surgery for skull base tumors is to remove the entire tumor. Radiation is almost always recommended after surgery for skull base tumors. The most important thing to know about radiation for chordoma is that a high enough dose must be used in order to effectively kill the tumor cells.

  • Guidelines for mobile spine and sacral tumors: The goal of surgery for mobile spine and sacral chordoma is to remove the entire tumor in one piece. Radiation plans can differ based on where the tumor is located. Radiation can be given after surgery, both before and after surgery, or instead of surgery if removing the tumor would be too risky or would cause side effects that are unacceptable to the patient. In some cases, if a sacral tumor is removed in one piece with wide margins, doctors will recommend that the patient not undergo radiation at all.

EU cross-border healthcare

EU citizens have the right to access healthcare in any EU/EEA country and to be reimbursed by their home country for treatment abroad. This is particularly important when dealing with a rare cancer such as chordoma, where treatment might not be available in your home country.

Directive 2011/24/EU on patients’ rights in cross-border healthcare sets out the conditions under which a patient can travel to another EU country to receive medical treatment and be reimbursed. It covers healthcare costs, as well as the prescription and supply of medications and medical devices.

Radiation across Europe

The goal of radiation therapy is to kill or stop the growth of tumor cells in the body. Radiation may play several roles in the treatment of chordoma, depending on the stage of the disease. Radiation with beams of charged particles, called particle therapy, is generally recommended for treating chordoma because it can be focused very precisely on the tumor. Two types of particles are commonly used: protons and carbon ions.

Recurrence and metastatic disease

More than half of all chordoma tumors grow back after initial treatment. This is called recurrence. When the tumor grows back at the original site, it is called a local recurrence. When the tumor spreads beyond the original site to other areas of the body, it is called metastasis.

There are a number of factors that will affect the treatment options available to you. Each patient’s situation is unique and must be evaluated on an individual basis. For any tumor location, the decision to have surgery alone, surgery plus radiation, or radiation alone should be made with your doctors based on your individual situation.

The CGCG has developed guidelines for the treatment of recurrent chordoma.

You can find the guidelines on these pages of our primary website:

The possibility of recurrence underscores the importance of long-term follow-up for chordoma patients. Regular imaging tests, such as MRI scans, are essential to monitor the treated area and detect any signs of tumor recurrence as early as possible.

There is currently no known cure for metastatic disease. However, several treatment options are available to control or temporarily stop tumor growth and relieve symptoms. These include debulking surgery, low-dose radiation, ablative therapies, and drug therapies.

Participate in a clinical trial

Most clinical trials for chordoma are designed for patients with locally advanced or metastatic disease. It is always important to talk to your chordoma experienced medical oncologist so they can help you determine which trial might be the best option for you based on your medical history and current situation. Clinical trials for chordoma often have a limited number of sites, and it might be necessary for you to travel to another EU country to access certain treatments. You can find trials on our drug therapy list.

If you are considering participating in a clinical trial in another EU country, it is a good idea to assess the financial implications, including the costs of travel, accommodation, and any additional fees not covered by the trial sponsors. Prepare for the logistical challenges of traveling and staying in another country for the trial duration (including language barriers, cultural differences, and the potential need for long-term stays). Seek support networks that can provide guidance, insights, and emotional support.

It is not always easy to participate in a cross-border trial. The EU-X-CT initiative seeks to enable and facilitate cross-border access to clinical trials for patients in Europe and make it easier for clinical investigators to enroll patients from other European countries in their clinical trials. Please consider sharing your experience by participating in their survey.

Cost of travel can pose a significant barrier for chordoma patients seeking treatment. The Chordoma Foundation offers financial assistance to patients participating in clinical trials, which may alleviate some of the financial burden. Patients enrolled in trials that are listed on the drug therapy options page of our main US website may qualify for this program.

Contact a Patient Navigator

Our Patient Navigators can answer your questions, provide information about treatment guidelines, help you find qualified doctors, and connect you with others in the chordoma community who have been through a similar journey.

“The Chordoma Foundation is the most extraordinary resource to have as a patient. Because the disease is so rare, it is easy to feel a bit lonely. But when you have the Chordoma Foundation bringing people together, it feels like a family with a shared experience. The Foundation provides amazing insights and advice, and is pioneering in its research. And the staff are fantastic, responding to questions quickly and professionally, and building relationships between experts and patients.”
Diggory, patient (UK)

Living with chordoma

With continued advances in cancer care and research, patients are living longer after being diagnosed with chordoma. Once treatment ends, chordoma survivors require regular follow-up visits and imaging tests to ensure that the chordoma has not returned and to manage any long-term side effects of treatment. These follow-up visits are critical to the early detection of recurrences and the management of any new health problems that may have arisen as a result of the chordoma or its treatment.

In addition to medical needs, it’s critical to support the emotional and psychological well-being of chordoma survivors. Patients may experience anxiety about the possibility of recurrence, or struggle with the lingering effects of their treatment, such as fatigue, pain, or changes in physical appearance. We have developed survivorship resources to help you: You can access in-depth information, resources, and guidance specifically tailored to address the side effects and quality of life issues that most commonly affect individuals after chordoma treatment in order to manage these issues and live a full life.