Clival and upper cervical side effects
Find out how to manage side effects like speech and swallowing issues, hearing loss, double vision, and endocrine system imbalances.
New diagnosis
These treatment guidelines were developed in June 2025 by the Global Chordoma Consensus Group based on all available medical and scientific evidence on chordoma diagnosis, treatment, and patient outcomes. The guidelines were published in the medical journal JAMA Oncology as a reference to help doctors provide better and more consistent care to their chordoma patients.
As part of our commitment to helping patients and caregivers make the most informed treatment choices, we have made those same recommendations available to you here in easy-to-understand language. Please read this information carefully and discuss it with your doctors.
How these recommendations were made
The Global Chordoma Consensus Group developed these recommendations to reflect the best available evidence and the shared expertise of leading chordoma specialists worldwide. In June 2025, more than 150 expert physicians met in Milan, Italy, to update and expand the first international consensus guidelines for primary, localized chordoma, which were published in 2015. The group included specialists from every field involved in diagnosing and treating chordoma, along with statisticians, molecular biologists, and patient representatives.
Before the meeting, the group carefully reviewed 305 chordoma research articles published over the previous 10 years. During the meeting, they developed recommendations based on the strength of the evidence. The resulting guidelines cover diagnosis; surgery and radiation planning for chordomas of the skull base (clivus), mobile spine, and sacrum; drug therapy; follow-up schedules; and supportive, palliative, and rehabilitation care.
The final guidelines were published in JAMA Oncology in July 2026. The information on this page is a plain-language version of those evidence-based recommendations that represent a shared agreement among these experts, describing the best way to care for primary (first-time), localized (in one anatomical area) chordoma.
Finding the right medical team
If chordoma is suspected or has been diagnosed, the most important next step is to be evaluated at a medical center with expertise in treating chordoma. Because chordoma is rare and complex, care should be provided by a multidisciplinary team of specialists who work together to coordinate treatment and have substantial experience treating tumors of the skull base and spine, including chordoma. These teams are typically found at larger academic hospitals, sometimes called referral centers, that see many patients with complex tumors.
Your care team should include specialists with experience diagnosing and treating chordoma in the following areas:
- Pathology
- Radiology
- Surgery
- Radiation oncology
- Medical oncology
- Palliative care and rehabilitation
It is also recommended that your doctors discuss your case in a multidisciplinary tumor board. This is a regular meeting where different specialists come together to review each patient’s case and develop the best treatment plan. As a patient, you benefit from the knowledge and experience of many experts instead of just one or two. This is very important for treating a complicated disease like chordoma.
Getting a diagnosis and preparing for treatment
Imaging can show the possibility of a chordoma, but a definitive diagnosis can only be made by a pathologist who examines a sample of your tumor tissue. Whenever possible, avoid having a biopsy outside of a referral center. If these procedures are not carefully planned and performed by an experienced team, they can make the tumor more difficult to treat and may increase the risk of tumor cells spreading. Recommendations for performing biopsies are located in each of the tumor location sections below.
Pathology
If your diagnosis was first made somewhere other than a center with chordoma expertise, it is strongly recommended that your tumor sample be reviewed and tested again by an experienced center.
Pathologists use immunohistochemistry (IHC) tests to confirm a chordoma diagnosis. IHC uses special stains to test for the presence of certain proteins in the tumor cells. For chordoma, one of the most important to look for is a protein called brachyury. All conventional chordomas are positive for brachyury, which helps tell chordoma apart from other conditions that can look similar, such as certain cartilage tumors (e.g., chondrosarcoma) or cancer that has spread from elsewhere in the body. Brachyury should be tested in all suspected cases of chordoma and is considered essential for confirming a chordoma diagnosis. Chordomas also typically stain positive for keratin, and sometimes for two other markers called EMA and S100. Benign Notochordal Cell Tumors also stain positive for brachyury.
The poorly differentiated subtype of chordoma loses expression of a protein called INI1, so IHC testing for INI1 should be done if the tumor might be poorly differentiated. In dedifferentiated chordoma, the aggressive component of the tumor is typically negative for brachyury and keratin, and sometimes loses INI-1 staining as well.
Genomic testing of newly diagnosed tumors can be performed for research purposes, but it isn’t necessary for guiding initial decisions on surgery and radiation.
Imaging
Chordoma tumors are typically detected through imaging tests, which show organs and other structures inside the body, including tumors.
Suspected chordoma
The way the tumor looks on imaging tests can tell a radiologist whether the tumor might be chordoma. Magnetic resonance imaging, also called MRI, is the best way to see a chordoma and how it is affecting the tissue around it, such as muscles, nerves, and blood vessels. Chordoma is best seen on an MRI with a setting called T2 weighted imaging.
Another imaging test called computed tomography, also known as CT or “CAT” scan, is recommended in addition to MRI if it is not certain whether the tumor is chordoma. CT scans of the chest, abdomen, and pelvis are also recommended.
Confirmed chordoma
Further imaging should be done to help plan your treatment and to better understand the size and exact location of the tumor. This process is often referred to as staging. You should have magnetic resonance imaging (MRI) of the main tumor site, an MRI of your whole spine, and a CT scan of your whole body. This is the best way to see a chordoma and how it is affecting the tissue around it, such as muscles, nerves, and blood vessels. A whole-body MRI should also be considered for pediatric patients or those who have a genetic syndrome associated with an increased risk of developing chordoma, such as tuberous sclerosis complex (TSC).
A positron emission tomography (PET) scan is not very useful for staging conventional chordoma, because this subtype doesn't consistently show up well on PET scans. However, this type of scan is recommended when a patient is first diagnosed with poorly differentiated or dedifferentiated chordoma, since these tumor types tend to show up more clearly on PET scans. It can also help detect whether the cancer has come back after complete surgical removal, or help guide a biopsy.
Depending on where the tumor is located, doctors need to check its relationship to different nearby structures:
- In the skull base: the opening at the base of the skull, the brainstem, a bony structure called the dens, the middle part of the skull base, the third ventricle and optic chiasm (structures near the center of the brain), the pituitary gland area, the sinuses, and the back of the throat.
- In the mobile spine: the fat around the spinal canal, the fluid-filled space around the spinal cord, the spinal cord itself, and the small openings where nerves exit the spine.
- In the sacrum: the openings where nerves pass through, the sciatic nerve, and the layer of tissue around the rectum (the mesorectal fascia) that usually protects it from being invaded by the tumor.
Treatment guidelines by tumor location
Your first treatments will have a big impact on both your quality of life after treatment and the chances of the tumor coming back.
Appropriate chordoma treatment requires a comprehensive, team-based approach at a specialized center that handles many cases of chordoma every year. The main treatment options are surgery and radiation. Your plan should take into account the features of your tumor (e.g., the location, the subtype, the area around the tumor, what nerves it is affecting) and your overall health. Decision-making should be shared between you and your team, balancing the goal of controlling the cancer and the impact that treatment could have on your quality of life. Rehabilitation specialists should be involved early in treatment planning, and your team should communicate consistently throughout your care.
These sections include the treatment you should have based on where your tumor is located. Click the arrows to expand each section.
Radiation dose recommendations for all tumor sites
Before radiation begins, your team will create a detailed map of exactly where to aim the radiation and where to avoid it. To build this map, they combine your CT images from before surgery with MRI images from both before and after surgery. Together, these images help your team outline the target volumes, which are the areas where the radiation will be delivered.
There are three target volumes:
- The first covers everywhere that could contain microscopic tumor cells (tumor too small to see on scans).
- The second is the tumor bed, the place where the tumor was. This area receives an extra, higher dose of radiation (a "boost").
- The third is any visible tumor that could not be removed during surgery. It also receives a boost.
When the tumor involves important nerves, your team adjusts these treatment areas to protect those nerves as much as possible.
While planning, your team also outlines the healthy structures near the tumor that are sensitive to radiation. Which structures should be protected depends on where your tumor is. This includes the brainstem and the nerves for vision near the skull base, the spinal cord along the spine, or the bowel and other organs near the sacrum. Each of these is given a safe dose limit, so the radiation stays within amounts known to keep them protected.
Chordomas resist radiation, so they need a high dose to be controlled. To give these high doses safely while protecting the healthy tissue around the tumor, doctors recommend either particle therapy (protons or carbon ions) or highly precise photon (X-ray) radiation. Some studies suggest people may live longer with particle therapy than with standard photon therapy, though the evidence is not yet firm, and more research is needed on carbon-ion therapy in particular.
Radiation dose is measured in units called Gray (Gy). For the tumor bed and any visible tumor, the recommended total is 70-79 Gy for protons and photons. This total is given in small daily amounts (called fractions) of about 1.8 to 2 Gy per session, spread over several weeks. Giving it a little at a time lets the healthy tissue recover between sessions. This approach is used for the two most common types of radiation: photons (standard X-ray radiation) and protons.
For carbon ions, slightly larger daily doses are given over fewer sessions, reaching a total of about 66 to 67.6 Gy for the tumor bed and any visible tumor, and 36 Gy or more for the area that may hold microscopic tumor cells.
Considerations for younger patients and rare subtypes
In children and adolescents, skull base chordomas are often more aggressive and complex. Radiation after surgery can improve the likelihood of controlling the tumor for these age groups.
For poorly differentiated tumors, drug therapy before surgery must be considered as an option. See the Drug therapies section for further guidance.
Long-term effects on function and on brain development in young patients are probably underreported, so standardized follow-up is important.
Interventional radiology
Image-guided treatments that kill tumor cells with heat or cold are done by interventional radiology specialists. Heat methods include radiofrequency ablation, microwave ablation, laser therapy, and high-intensity focused ultrasound. Irreversible electroporation (IRE) uses electricity, and percutaneous cryoablation (PC) uses extreme cold.
Radiofrequency ablation can work well for small tumors (less than 2 cm) but does not allow real-time monitoring. Percutaneous cryoablation can treat irregularly shaped tumors using several freezing probes. It also lets doctors watch the frozen zone in real time.
The evidence for percutaneous cryoablation in chordoma comes from small studies looking back at past cases, mostly in the sacrum and tailbone area, showing pain relief and local control for small recurrences (less than 5 cm). Therefore, cryoablation is not a substitute for wide surgical removal, but it may be considered when surgery or radiation are not possible. It should be used cautiously since its long-term ability to control the tumor is still unknown.
This type of treatment is often limited by nearby critical structures, including blood vessels and nerves, the covering around the spinal cord, organs such as the bowel, and sensitive tissue such as in the nasopharynx. Some protective measures may lower this risk. Based on early experience at referral centers, freezing the tumor before removing it may make the jelly-like chordoma tissue firmer, which could reduce the spilling and spreading of tumor cells during surgery. However, there is not yet enough evidence for a recommendation to be made on this approach.
Any type of interventional radiology approach should be carefully and thoroughly discussed with your doctors.
Drug therapies
Standard chemotherapy has shown very little benefit for conventional chordoma. For that reason, drug therapy is not recommended as the first treatment if your tumor is in one location and can be treated with surgery and/or radiation.
Poorly differentiated chordoma (PDC) and dedifferentiated chordoma (DC) are more aggressive subtypes and may respond to chemotherapy. When PDC is confirmed, chemotherapy should be considered, possibly before surgery. Combinations of several drugs—including anthracyclines, platinum agents, irinotecan, alkylating agents, vincristine, and etoposide—have been used and show response rates above 50%. There is not much evidence for treating DC with drug therapies, but chemotherapy may be considered since DC can resemble a high-grade sarcoma.
Because the benefit is uncertain and side effects are possible, drug therapy should be considered only if you are in good enough overall health.
Follow-up care
Conventional chordoma recurs in the same area in up to 50% of patients after an R0 resection. This rate is higher after a partial resection. Recurrences can happen up to 15 years after treatment, so long-term follow-up is essential. You should have an MRI of the original site every 6 to 12 months for the first 4 to 5 years, then every 1 to 2 years for at least 15 years. A chest CT and whole-spine MRI can be used to watch for distant spread and to check on any recurrence.
Poorly differentiated and dedifferentiated chordoma are more likely to spread to other areas of the body, so follow-up should match the schedule used for high-grade bone sarcoma. This means an MRI of the original site plus checks for distant spread (whole-spine MRI and chest imaging) every 2 to 3 months for 2 years, then every 3 to 6 months until year 5, then once a year for at least 10 years. In children, adolescents, and young adults, chest X-ray and low-dose CT can be alternated to limit radiation exposure.
If you had a skull base chordoma, a referral to an endocrinologist and hormone testing are recommended, because the pituitary gland can be affected by surgery or radiation. Appropriate treatment for any imbalances should be consistently maintained. Vision (visual field) testing is also recommended, depending on the radiation dose your optic nerves received.
Follow-up recommendations from the U.S. National Comprehensive Cancer Network
In addition to the Global Chordoma Consensus Group guidelines, the U.S. NCCN Bone Cancer Guidelines for chordoma recommend a CT scan of the chest every 6 months for 5 years, and then annually thereafter, to see if chordoma has spread to the lungs.
Palliative care and rehabilitation
Chordoma and its treatment can cause a range of impairments. Common issues include pain, muscle weakness, numbness or altered sensation, difficulty with movement or balance, fatigue, and problems with the cranial nerves (which control functions like facial movement, swallowing, and vision), as well as difficulty swallowing, speech problems, and bowel, bladder, or sexual difficulties. Two kinds of specialists can help you manage these: rehabilitation medicine specialists and palliative care specialists. Their roles overlap, and many patients benefit from both.
Rehabilitation medicine
A rehabilitation medicine specialist (also called a physiatrist) focuses on restoring function and independence. Care should begin with a thorough assessment to identify what's causing each symptom, pinpoint any nerve, muscle, or bone problems, and understand how these affect your daily life. From there, the physiatrist sets rehabilitation goals together with you and the rest of your treatment team, taking into account whether symptoms might worsen, any activity restrictions needed for safety, and what you and your caregivers hope to achieve.
Rehabilitation may include:
- Physical and occupational therapy tailored to your needs around strength, mobility, balance, and daily activities, including any equipment that might help, such as a walker or wheelchair. This can include specialized therapy for balance (vestibular), vision, or pelvic floor issues.
- Speech therapy for trouble swallowing or communicating.
- Braces to help with positioning, stability, and compensating for weakness.
- Bowel and bladder management programs and personalized exercise plans.
Rehabilitation can take place in the hospital, in an outpatient clinic, or at home, depending on what you need.
Palliative care
A palliative care specialist focuses on relieving symptoms and improving quality of life at any stage of the disease. Pain is a common and often difficult symptom, because chordoma affects the bones and can compress or grow into nearby nerves, and because treatment itself can cause side effects. A palliative care specialist can carry out a thorough pain assessment to understand what's causing it and guide the right treatment, and can also help manage other burdensome symptoms such as fatigue. They can also help identify which other specialists you might need to address specific side effects, and coordinate that care. Beyond physical symptoms, palliative care can also connect you with counseling, complementary therapies, and support for you and your caregivers. They can also help you and your loved ones navigate difficult decisions and ensure that your treatment continues to reflect what matters most to you.
Working as a team
Throughout your care, your rehabilitation and palliative team should watch for other treatment-related health problems and for any signs that the disease is returning, adjusting your plan along with the oncology team as needed.
Dealing with side effects
Treatments for chordoma and sometimes the tumor itself can often cause side effects and other issues that affect your quality of life. Our resources can help you understand these challenges and learn how to manage them.
Connect with the chordoma community
Support from someone who's been there can make all the difference.
A rare disease like chordoma can be lonely, so we provide ways for patients, survivors, caregivers, and co-survivors to connect with one another.
Peer Connect
Peer Connect is a free, confidential peer-to-peer support program that connects anyone touched by chordoma with another person whose experiences with chordoma are similar. Trained Peer Guides are available to support chordoma patients who are newly diagnosed, patients in active treatment, survivors, caregivers, family members, or friends.
Connect with a Peer Guide
The information provided herein is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your or your child’s physician about any questions you have regarding your or your loved one’s medical care. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.