How Is Chordoma Treated?

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Treatment for chordoma—a rare bone cancer causing tumors on the spine or base of the skull—will depend on the tumor's size, location, and whether the cancer has spread. The main treatment options usually include a combination of surgery to remove the tumor and radiation therapy to kill any remaining cancer cells.

The goal is to treat chordoma quickly before it spreads or causes damage to other parts of the body. Unlike other cancers, chordoma does not respond well to chemotherapy, but scientists are investigating other potential treatments, like targeted drug therapy and immunotherapy.

This article discusses the effective treatment options available for chordoma and provides information about the growing research into new alternatives for chordoma treatment.

Scans of a person's spine

 HadelProduction / Getty Images

Surgery

After a chordoma diagnosis, the first treatment option that healthcare professionals will consider is surgery to remove the tumor.

By surgically removing the tumor, along with a small amount of the surrounding tissue containing cancer cells, there is a lower chance that chordoma will return in the future. If the chordoma can't be removed completely, the goal is to remove as much as possible.

It can be difficult and complex to treat chordoma tumors with surgery, because they often grow closely to sensitive areas like the spinal cord, brain, nerves, and arteries. The type of surgery done will depend on exactly where the tumor is located, including:

  • Spinal chordoma: For tumors located on the spine or near the tailbone (sacrum), the surgery will likely involve making a surgical opening on the skin to remove the tumor and some of the nearby tissue. If it's too close to the spinal cord or nerves, it may not be possible to take the entire tumor out safely.
  • Skull base chordoma: Tumors that grow at the base of the skull (near where the head meets the neck) can be a little trickier to treat with surgery because they're so close to the brain and spinal cord. Surgeons may use what's known as an endoscopic surgery to remove as much of the tumor as safely as possible. This can be done using instruments that enter through a tiny incision in the back of the nose or the skull.

Recurrence

Keep in mind that it's possible for chordoma to return after surgical treatment, which is why it's important to continue follow-up care with your medical team in the weeks, months, and years ahead.

Radiation Therapy

Radiation therapy is another main treatment option for chordoma. It involves using beams of high-energy rays of radiation to kill cancer cells. These rays are targeted at the part of your body where the chordoma is located to destroy or slow any cancer cell growth.

Your healthcare provider or oncologist may recommend radiation therapy in the following situations:

  • Before surgery to shrink a chordoma and make it easier to remove.
  • After surgery to kill any remaining cancer cells and lessen the chances that the chordoma will return.
  • As the main treatment if surgery isn't an option due to the chordoma's location.

For chordoma, healthcare providers will usually use precise, targeted types of radiation, such as proton beam therapy and intensity-modulated radiation therapy (IMRT), to accurately target specific locations, which may result in less damage to surrounding tissues.

A radiation session usually takes up to 30 minutes, and chordoma patients may have multiple sessions over the course of several weeks. The radiation procedure itself should be painless. Common short-term side effects include fatigue, hair loss, and skin changes.

Other Treatment Considerations

In addition to the tumor's size and location, your oncologist (a medical doctor specializing in diagnosing and treating cancer) may take the following factors into consideration when coming up with a treatment plan:

  • Your age
  • Potential side effects
  • Any other health conditions you may have

Prescriptions

There are currently no prescription medications to treat chordoma, but your healthcare provider may prescribe certain medications to help with painful symptoms.

Chronic pain is a common side effect for chordoma patients. It can be caused by pressure on nearby nerves from the tumor itself, or the pain is sometimes due to nerve damage from surgery.

Depending on the kind of pain you're experiencing and how severe it is, controlling your pain may take a combination of medications, including:

  • Prescription pain relievers, known as opioids, to help control chronic pain
  • Corticosteroids, like cortisone and prednisone, to reduce pain and inflammation in the body
  • Pain-relief patches applied to the skin that contain a prescription-strength local anesthetic like lidocaine
  • Nerve blocks that disrupt the pain signal from the nerve to the brain through an injection of a local anesthetic to the nerve area

Risks of Opioid Use

Opioid medications can be effective and safe for certain conditions when used under close medical supervision. But they can also interfere with other medications, cause severe drowsiness, and potentially lead to addiction. Your healthcare provider will discuss these risks and benefits with you.

Home Remedies and Lifestyle

There are no specific home remedies or lifestyle changes that can treat chordoma. But in general, experts note that taking good care of yourself through diet, exercise, and self-care has the potential to improve overall health and well-being in cancer patients.

Every person's journey with chordoma is different, so it's a good idea to talk to your healthcare provider about whether any of the following tips would be safe, helpful additions to your chordoma treatment plan:

  • Diet: Experts recommend getting the necessary nutrients through a well-balanced diet rather than supplements, which may potentially interfere with cancer treatments. Check with your healthcare provider about any nutritional or diet adjustments needed before, during, or after chordoma treatment to make sure your body stays as nourished as possible.
  • Exercise: General guidelines encourage cancer patients to take part in regular physical activity if they're able to. This could be exercising several times a week for at least 10 minutes each time, including light cardio, resistance training, and stretching.
  • Physical medicine and rehabilitation: Physical therapy or occupational therapy can help with pain and mobility, particularly after chordoma surgery. Your medical team will likely create a rehabilitation plan to help maximize your recovery from chordoma treatment.
  • Integrative therapies: Integrative medical care combines conventional medical treatment with complementary and alternative medicine (CAM) therapies that have been known to be safe and beneficial for people with cancer and other conditions. It includes therapeutic options such as acupuncture, massage therapy, yoga, qigong, biofeedback, meditation, and mental health counseling, among others. While there is little evidence to show that these treatments can directly help treat chordoma or other forms of cancer, they may help patients cope with the mental, emotional, and physical toll such diseases can take.

Experimental Treatments

Currently, the Food and Drug Administration (FDA) has not approved any medications for treating chordoma, mostly because there aren't any large scientific studies to provide evidence of success. That said, some newer, experimental medications have been shown to be effective in treating chordoma during clinical trials.

Targeted Drug Therapy

Targeted drug therapies are medications that specifically target cancer cells, controlling how they grow, divide, and spread. They're often less harmful to the surrounding normal, healthy cells. These drugs are typically taken as daily pills, and may come with side effects like diarrhea, nausea, muscle pain, or fatigue.

While some targeted therapy drugs have been approved for other bone cancers, they haven't been approved by the FDA for the treatment of chordoma. Some healthcare providers may use these promising drugs off-label (before they've been approved for such use), particularly for tumors that have returned after surgery or radiation.

Research is currently ongoing for some targeted therapies such as:

  • Gleevec (imatinib)
  • Sprycel (dasatinib)
  • Sutent (sunitinib)

Immunotherapy

Immunotherapy is a cancer treatment that uses your own immune system to fight cancerous cells.

Broadly speaking, this type of treatment use substances that naturally occur in the body or substances altered in the laboratory to positively change the body's immune response. Some of the side effects include skin reactions, flulike symptoms, muscle aches, and shortness of breath.

While it's not yet approved to treat chordoma, immunotherapy research and clinical trials are currently underway. Some studies have shown that certain immunotherapy drugs can be combined with specific types of radiation to effectively treat chordoma.

Other findings have suggested that immunotherapy may be particularly helpful for chordoma patients who have not responded well to surgery or radiation.

Summary

Chordoma is a rare type of bone cancer that forms tumors on the spine or base of the skull. Treatment options will vary based on the tumor’s size, location, and whether the cancer has spread. Most chordoma patients need surgery to remove the tumor, radiation therapy to kill off any remaining cancer cells, or a combination of the two treatments.

There are currently no medications or lifestyle adjustments that have been proven to treat chordoma. Researchers are investigating other promising therapeutics—like targeted drug therapy and immunotherapy—that may be helpful treatment options in the future.

A Word From Verywell

Chordoma is an extremely rare type of bone cancer, with roughly 300 new cases diagnosed in the United States every year. This is part of the reason why it's difficult for researchers to find enough patients for clinical trials in order to develop more treatment options. That said, you should know that experts are working hard to come up with newer treatments for chordoma patients, particularly for those cases in which surgery or radiation may not be effective.

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