Treatment guidelines for newly diagnosed chordoma
Finding the right medical team
If chordoma is suspected or has been diagnosed, the most important thing to do is find a medical center with expertise in treating chordoma patients. Chordoma is a rare disease that requires expertise to treat properly. It is important to have a multidisciplinary medical team that includes multiple specialists who work together to coordinate your care. All members of the care team should have substantial experience treating tumors of the The 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. and spine, including chordoma.
Your care team should include specialists with experience diagnosing and treating chordoma in the following areas:
- Radiation oncology
- Medical oncology
- Care given to improve the quality of life of patients who have a serious or life-threatening disease.
It is also recommended that your doctors discuss your case in a multidisciplinary A group of different types of specialists within a medical center who meet regularly to review each patient’s situation and make treatment recommendations.. 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.
To help you find the best care possible we have organized a list of chordoma experts you can search by specialty, location, and key terms.
Getting a diagnosis
If you’ve been told you might have chordoma, it is important to be evaluated by a Treatment 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. of specialists who have substantial experience treating chordoma, even before you know for sure if you have it. Teams with this experience are typically only found at larger hospitals, sometimes called referral centers, which see large numbers of patients. You should avoid having a A procedure that uses a needle to remove a small tissue sample from the tumor to be tested in order to make a diagnosis. or surgery to confirm the diagnosis outside of a A hospital, treatment center, or network of treatment centers where doctors have expertise in particular diseases. because, if not done properly, these procedures can cause the chordoma to spread.
Get the best care possible
No matter the stage of your disease, there are some important points to keep in mind while you are learning about your options and making decisions.
Chordoma tumors are typically detected through imaging tests, which show organs and other structures inside the body, including tumors. The way the tumor looks on imaging tests can tell a radiologist whether the tumor might be chordoma.
When a chordoma is suspected, you will need A type of imaging scan that is used initially to help diagnose chordoma, as well as during follow up to check for recurrence or metastasis., also called MRI, to help doctors make a diagnosis and plan for treatment. 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. No matter where the tumor is located, an MRI of the entire spine should be performed to see if the tumor may have spread to or developed in other areas of the spine. Chordoma is best seen on an MRI with a setting called T2 weighted imaging.
Another imaging test called A type of imaging scan that is used to help diagnose chordoma and can also be used to help guide the needle during a biopsy. They are also referred to as CT scans or “CAT” scans., 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.
In some cases, doctors may order a positron emission tomography, or PET, scan. These are usually whole-body scans that look for widespread tumor activity. PET scans provide information about your body’s metabolic function; for cancer specifically, PET scans pick up on tumors that are rapidly using glucose (sugar). Cancer cells metabolize much more glucose than regular cells, so tumors “light up” on the scan. Because chordoma cells typically grow slowly, however, they are not always picked up as well by PET scans, so they are not always used in chordoma diagnosis or treatment.
These images should be interpreted by a radiologist who has experience diagnosing bone tumors.
While imaging studies can show the possibility of a chordoma, a definitive diagnosis can only be made by a pathologist who examines a sample of tumor tissue under a microscope.
For this reason, your medical team may consider taking a small sample of tissue from the tumor, called a biopsy, prior to surgery. However, biopsies are not recommended if the tumor cannot be reached safely or when there is a high risk of spreading tumor cells. For sacral and The 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. tumors, a A 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 and should be done from the back.
Trocar CT-guided biopsy uses a CT scan to precisely direct the biopsy needle to the correct location. The biopsy needle is enclosed in a tube to keep tumor cells from spreading along the path of the needle — this is often called Tumor cells deposited along the path of a biopsy needle or left in areas around the tumor during surgery, which will eventually grow into tumors. Seeding is a major concern for chordoma tumors and care should be taken to prevent it.. Check with your doctors to make sure they will use this method if a biopsy is planned.
If you have a biopsy before surgery, it is recommended that your surgeon remove the tissue around the area of the biopsy during surgery in order to remove any chordoma cells that might have spread when the biopsy disturbed the tumor.
Tissue samples should be evaluated by a pathologist who has experience diagnosing bone tumors. Your pathologist should test your tumor tissue for the presence of a protein called A gene that makes a protein, also called brachyury, that is present at high levels in nearly all chordoma tumors.. Nearly all chordomas have high levels of brachyury, which makes it helpful for diagnosis.
|The Chordoma Foundation Medical Advisory Board suggests that chordoma patients under 35 years of age, or whose tumors are growing unusually fast, have their tumors tested for loss of A protein that regulates the growth of certain tumor cells. Poorly differentiated chordoma tumors are characterized by loss of INI1. Also called SMARCB1., either through immunohistochemistry (IHC) or genomic testing. If tissue is not available for testing, the possibility of a biopsy to obtain tissue for this testing should be discussed with a doctor who has experience treating chordoma, carefully weighing the potential risks of biopsy. Interpretation of the testing should be done by a pathologist who has experience diagnosing chordoma.|
After you are diagnosed with chordoma you will most likely need to have surgery, radiation, or both. These treatment methods have the potential to cure some chordoma patients if done properly. The Consensus Group’s recommendations vary based on where the tumor is located. Use the buttons below to find recommendations for surgery and radiation for your tumor location.
If you have already had initial treatment outside of a referral center
Regardless of what initial treatment you may have had, it is still very important to be evaluated at a referral center as soon as possible. In particular, it is a good idea to have a sample of your tumor sent to a referral center where an expert pathologist can confirm the diagnosis.
Individualized treatment plan
Each patient’s situation is different, so you should talk with your doctors about the course of treatment that makes the most sense for you. Your doctors can help you understand all the treatment options that are available to you, and create an individualized treatment plan based on your choices.
Getting multiple opinions from doctors who have experience with chordoma can help you make the most informed decision about your treatment. The Chordoma Foundation Doctor Directory provides information for doctors who have this experience.
A Chordoma Foundation Patient Navigator can help you find doctors who have experience with chordoma. Our Patient Navigators are available Monday through Friday from 8 AM to 5 PM Eastern Time.
The information on this page was developed by the Chordoma Foundation in consultation with members of the Chordoma Global Consensus Group. We would like to thank the members of the Chordoma Global Consensus Group for providing their expertise in the development of the original consensus guidelines and their review of this educational content.
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 loved one’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.