Q&A: Chordoma, a one in a million bone cancer

When you have a rare cancer that only affects one in a million people, it can be difficult to find the answers and information you need. That’s why we sat down with spinal neurosurgeon Laurence Rhines, M.D., and skull base neurosurgeon Shaan Raza, M.D., to discuss chordoma, a rare, malignant tumor diagnosed in about 300 people each year in the United States.

What is chordoma?

Chordoma is a bone cancer that belongs to the sarcoma family. About 50% of chordomas occur in the sacrum, the bone at the base of the spine that forms part of the pelvis. About 30% occur in the skull base, the area behind the eyes and nose that slopes down to form the “floor” of the skull. The rest are found along the spine.

What are common chordoma symptoms?

The symptoms vary, depending on the tumor’s location. The spine and skull base contain important nerves. When a tumor presses on these nerves, it can cause pain or neurological issues. For sacral chordomas, neurological symptoms frequently include bowel, bladder and sexual dysfunction. For skull base chordomas, common symptoms include double vision, headaches, neck pain and trouble swallowing.

How is chordoma diagnosed?

An MRI scan can help rule out other tumor types, but a tissue sample is needed for a definitive chordoma diagnosis. If your tumor is in the spine, an interventional radiologist will typically perform a CT-guided core biopsy to obtain a tissue sample. Most skull base chordomas grow in a bone called the clivus. This area is difficult to access for biopsy, so a skull base chordoma diagnosis cannot be confirmed until after your neurosurgeon accesses the tumor during surgery, which is also the first step for treatment.

How is chordoma typically treated?

Chordoma treatment begins with surgery. When the entire tumor is successfully removed with surgery, patients statistically live longer and the tumor is less likely to recur, or grow back. The type of surgery and next treatment steps depend on the location of the tumor.

• In the spine: The goal is to not only remove the entire tumor, but to remove it in one piece. This is called an en bloc resection. Normally, a spine surgeon’s No. 1 priority is to protect your neurological function. With a chordoma, however, patients have the best long-term outcomes if the tumor is removed in one piece, even if that means sacrificing nerves to do so. It’s important to have an experienced neurosurgeon who can realistically explain the potential neurological consequences of the surgery upfront, so you can make an educated decision. When a spinal chordoma is “broken” during the surgery and not removed in one piece, it’s more likely to recur and cause further neurological complications. We work very hard with our patients to help them back to a high a quality of life after surgery.
  
  At MD Anderson, radiation therapy is most often used for a spinal chordoma if the tumor recurs or if the tumor is not removed in one piece. 
  
  
• In the skull base: Removing a tumor from the skull base may take a combination of open and minimally invasive techniques, which use the nasal passages to reach the tumor. Complete removal of the tumor and any invaded bone is known as gross total resection. If your entire tumor was not successfully removed with the first surgery, consider getting a second opinion on removing the remaining tumor before staring radiation. 
  
  After surgery, most skull base chordoma patients receive proton therapy. Chordomas require a high radiation dose, and proton therapy provides the most precise, concentrated dose while minimizing disruption to surrounding normal tissue. 

Currently, there isn’t a very effective chemotherapy option for newly diagnosed chordomas. If your chordoma recurs, your doctor may suggest trying a chemotherapy that has worked for other types of cancer or that is part of a clinical trial. Additional surgery and radiation therapy may also be used to treat a chordoma recurrence.  

How common is a chordoma recurrence?

About 50% of skull base chordomas and 30-40% of spinal chordomas recur. After treatment, we follow patients closely with regular MRI and CT scans to catch any recurrence as soon as possible. When a chordoma grows back, it usually does so near the location of the original tumor, within five to seven years of the initial diagnosis. In some cases, the cancer will spread to other parts of the body.

What research is currently underway for chordoma?

We currently don’t know which chordoma patients are at high-risk for recurrence. We’ve been gathering fresh frozen tissue from spinal chordomas for three years, alongside detailed clinical histories. Our goal is to find out if there are molecular clues to how the tumor will behave.

Because this is a rare and typically slow-growing disease, we’re collaborating with other cancer centers around the world to share data, so we can hopefully make progress faster.

What’s your advice for newly diagnosed chordoma patients and caregivers?

If you’re researching treatment options, look for a center with multidisciplinary expertise in treating chordomas. Know that you have reason to be hopeful. This is a new and exciting time for chordoma research, and the future is bright.  

To learn more about chordoma, register for the Chordoma Community Conference at MD Anderson on Saturday, Oct. 28. Any chordoma patient, friend or family member is welcome to attend to learn about the latest in disease management and research in progress, network with others affected by chordoma and ask the physicians questions outside of the clinic.

Request an appointment at MD Anderson online or by calling 1-877-632-6789.