Systemic Therapy Table
To assist chordoma patients who are considering systemic therapies, we have created a resource designed to clarify the options that are currently available through clinical trials or off-labelThe practice of prescribing drug treatments that are not approved by government agencies to treat a particular disease. Doctors are allowed to prescribe drugs off-label if they believe it is in the best interest of the patient. prescription. This resource is meant to make it easier for chordoma patients and caregivers to research treatment options.
The table below includes therapies corresponding to all mechanisms of action our Medical Advisory Board (MAB) has identified as relevant to chordoma treatment. A mechanism of action is the way a systemic therapyThe use of drugs that spread through the body to kill cancer cells. Also called chemotherapy or drug therapy. works to attack tumor cells. In the case of targeted therapies, for instance, the mechanism of action is to block a molecular target that the tumor depends on for survival. For example, the mechanism of action of an EGFR inhibitor is to block the EGFR protein, which would be referred to as the drug’s target.
How to use the Systemic Therapy Table:
- We encourage patients who are considering systemic therapies to show this table to their doctors to help guide discussions about treatment options. Click here to view the table as a spreadsheet that you can download or print out to take with you to your appointments.
- Follow the links under “Mechanism of action” to see a summary of the published supporting evidence.
- Follow the available links under “Open clinical trialResearch studies involving human subjects that are done to test whether a treatment is safe, and how well it will work to treat a specific disease.” for more information about those trials.
- Visit the Systemic Therapy for Chordoma page to learn more about when these treatments are recommended.
| Targeted Therapy | EGFR inhibitor | afatinib | Gilotrif | Yes | Yes |
| cetuximab | Erbitux | Yes | |||
| gefitinib | Iressa | Yes (outside the U.S.) | |||
| erlotinib | Tarceva | Yes | |||
| lapatinib | Tykerb | Yes | |||
| CDK4/6 inhibitor | palbociclib | Ibrance | Yes | Yes | |
| mTOR inhibitor | sirolimus | Rapamune | Yes | ||
| everolimus | Afinitor | Yes | |||
| PI3K inhibitor | taselisib | Yes | |||
| EZH2 inhibitor | tazemetostat | Yes | |||
| PDGFR inhibitor | imatinib | Gleevec | Yes | ||
| sorafenib | Nexavar | Yes | |||
| sunitinib | Sutent | Yes | |||
| VEGF inhibitor | regorafenib | Stivarga | Yes | Yes | |
| pazopanib | Votrient | Yes | |||
| bevacizumab | Avastin | Yes | |||
| Immune Checkpoint Inhibitor | PD-1/PD-L1 inhibitor | atezolizumab | Tecentriq | Yes | |
| avelumab | Bavencio | Yes | |||
| durvalumab | Imfinzi | Yes | Yes | ||
| nivolumab | Opdivo | Yes | Yes | ||
| pembrolizumab | Keytruda | Yes | Yes | ||
| M7824 | Yes | ||||
| Therapeutic Vaccine | Anti-brachyury vaccine | GI-6301 | Yes (on hold) | ||
| Autologous tumor vaccine | MVX-ONCO-1 | Yes | |||
| Intratumoral injection | INT230-6 | Yes |
All medical publications that contain case reports of or clinical trial data on chordoma patients’ responses to systemic therapy can be accessed in this publications table.
Chordoma Foundation Medical Advisory Board recommendationsAlthough each patient’s situation is unique, in general, our Medical Advisory Board (MAB) recommends that patients considering systemic therapy treatment options do so in the following order of priority, in consultation with a chordoma-experienced medical oncologist: 1. Chordoma-specific clinical trials 2. Other relevant clinical trials recommended by an experienced physician 3. Off-label therapy with evidence of clinical benefit for chordoma patients The Chordoma Foundation is a resource for clinical trials. Our Clinical Trials Catalogue lists all chordoma-specific clinical trials, as well as trials that our MAB has determined are relevant to chordoma patients based on what we know about the biology of chordoma in relation to the type of drug being studied in the trial. Listings in the Catalogue include information on the treatment being studied, the locations of trial sites, and links to learn more about the trial. Read more about clinical trials for chordoma » Search our Clinical Trials Catalogue » |
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Criteria for inclusion in this table:
1. Mechanism of action that the Chordoma Foundation Medical Advisory Board has recommended be explored in a clinical trial for chordoma.
2. Any mechanism of action that has been studied in a Phase 2 trial for chordoma.
3. The therapies listed for each mechanism of action are either currently being studied in a clinical trial that is enrolling chordoma patients or available off-label.
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.