Pain management
Pain is very common among people who have been diagnosed with any type of cancer. It is important to know that pain can be managed and it is your right to get treatment for it.
Advanced and metastatic disease
These treatment guidelines were developed by the Chordoma Global Consensus Group based on all available medical and scientific evidence on chordoma treatment and patient outcomes. The guidelines were published in the medical journal Annals of 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. Please read this information carefully and discuss it with your doctors.
Understanding advanced and metastatic disease
Chordomas are considered advanced when an isolated recurrence cannot be treated with high-dose radiation or when the recurrence is multifocal. Multifocal recurrences involve multiple tumors in the area of the original tumor location. Metastatic chordoma is one or more tumors that have spread to other parts of the body.
Advanced disease is very unlikely to be cured and currently, there is no known cure for metastatic disease. However, multiple treatment options are available to control or temporarily stop tumor growth, as well as to alleviate symptoms. These include debulking surgery, low-dose radiation, ablative therapies, and drug therapies. With the right care, your tumor may be controlled for many years while also maintaining a good quality of life.
Advanced disease is very unlikely to be cured and currently, there is no known cure for metastatic disease. However, multiple treatment options are available to control or temporarily stop tumor growth, as well as to alleviate symptoms. These include debulking surgery, low-dose radiation, ablative therapies, and drug therapies. With the right care, your tumor may be controlled for many years while also maintaining a good quality of life.
Learn more about drug therapy options
Finding the right medical team
When confronting advanced or metastatic disease, finding the right medical team is a critical first step. Depending on your situation, the medical center where you were treated initially may or may not be best suited to diagnose and treat a recurrence.
It is important to be evaluated and treated by a multidisciplinary team of specialists who have substantial experience treating chordoma. Teams with this experience are typically only found at larger hospitals, sometimes called referral centers, which see large numbers of patients.
Your care team should include specialists with experience diagnosing and treating chordoma in the following areas:
- Radiology
- Pathology
- Surgery
- Radiation oncology
- Medical oncology
- Palliative care
Biopsy for metastatic disease
If your tumor has spread to other areas of the body, a biopsy may be recommended, if it can be done safely. A biopsy may also be done if your tumor is growing unusually fast or if your doctors think you may have developed a new form of cancer that is not chordoma. A core-needle biopsy is recommended and should be performed by doctors who have experience with chordoma. The tissue sample should be examined by a pathologist with experience diagnosing chordoma.
If your tumor is growing quickly, the tissue sample should be analyzed to determine if the tumor has become poorly differentiated or dedifferentiated, both of which may require different treatment approaches. Poorly differentiated chordomas often lose expression of a protein called INI1, and sometimes dedifferentiated and conventional chordoma can as well.
If your doctors are unsure whether your tumor has spread and you are not experiencing any symptoms, it may be possible to wait and do imaging of the area again after a period of observation rather than performing a biopsy.
From the Chordoma Foundation Medical Advisory Board
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 INI1, 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.
Determining treatment options
There are a number of factors that will affect what treatment options are available to you. Each patient’s situation is unique and must be evaluated individually. Decisions about treatment should balance side effects, quality of life, and how well the treatment is expected to work. This will help ensure that you get the best treatment for your tumor with as little impact on your quality of life as possible.
This flowchart illustrates the possible treatment options for advanced and metastatic disease as discussed in the tabs below.
Treatment for advanced and metastatic disease
How do I decide?
Every patient’s situation is unique. If you have advanced or metastatic disease, it is important to discuss all of these options in detail with your medical team and your family, weighing the risks and benefits of each option for your situation. The treatment options listed here involve various types of doctors, so it is important to consult with doctors who specialize in these treatments and have experience treating chordoma patients. For some patients, just one type of treatment might be the best decision, while for others a combination of treatments might provide the best option.
Follow-up care
After you complete treatment, you will need to maintain a consistent schedule of follow-up imaging to check whether the tumor has returned or spread to other areas.
It is important for a chordoma expert who knows your case to review your follow-up scans and compare them to your previous ones. Depending on the treatment you’ve had, this monitoring may be done by your surgeon, radiation oncologist, or medical oncologist. If you traveled for your treatment and it would be difficult for you to travel back to your treating doctor for each follow-up appointment, discuss this with your doctor to make a plan for having the scans done locally and the images sent to your doctor for review.
The Chordoma Global Consensus Group guidelines state that you should have an MRI every 6 months for the first 5 years after treatment. The MRI should look at the area of the original tumor as well as any areas where it could spread. After 5 years, you should have an MRI at least once a year for 15 years.
Additionally, the U.S. National Comprehensive Cancer Network guidelines 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. Some experts suggest the MRI include the whole spine, and that CT scans of the pelvis and abdomen be done annually as well. It is important to talk with your doctors about what monitoring you need after treatment.
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 about how to manage them.
Emotional wellness
It's important to care for your mental and emotional health along with your physical health.
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.
Mobile spine and sacral side effects
Find out how to manage side effects like loss of mobility, bladder and bowl incontinence, and sexual dysfunction.
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.