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

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,
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
or by calling 1-877-632-6789.