Chordomas

Chordomas grow from remnants of cells that form the backbone in utero. They can occur anywhere along the spine but are most common in the sacrum (a bone at the bottom of the spine that forms a connection between the spine and the pelvis) and in the skull base (the bottom or floor of the skull). 

Chordoma Symptoms
Fortunately, these tumors grow quite slowly, but as they become large enough to press on nerves, they can cause pain and neurological problems. Common symptoms of sacral chordomas include loss of bowel, bladder, and sexual function. Skull base chordomas can cause headaches, facial and neck pain, double vision, swallowing and speech problems, and numbness.

Your chordoma care team will perform a thorough physical examination, take a detailed medical history, and review any existing imaging scans or test results. From there, your doctors will likely order additional tests, which may include:

Imaging Scans
X-ray, MRI, CT, PET, and bone scans as well as cerebrospinal fluid collection can help to rule out other types of cancer and identify additional tumors in other parts of the body.

Needle Biopsy
A tissue sample taken from the tumor is needed for a definitive chordoma diagnosis. Our surgical oncologists work closely with radiologists to determine how to safely access the tumor -- aided by CT guidance -- and remove a sample without contaminating nearby tissue. Then the sample is reviewed by our pathologists. Many skull base chordomas are difficult to access for biopsy, so a diagnosis may not be confirmed until surgery.

Staging
Together, these tests will help your oncologist stage your tumor, which means determining the tumor’s size and whether the cancer has spread (or metastasized) to other parts of your body. Fortunately, chordomas are usually caught before they metastasize.

After evaluation, your chordoma care team will meet to discuss your test results and make recommendations for a treatment plan. Chordoma treatment typically involves both surgery and radiation therapy.

Surgery
Chordoma surgeries are challenging because of the locations of these tumors. The goal for both skull base and sacral chordomas is to remove as much of the tumor as possible and all in one piece, if possible, which means part or all of a bone may need to be removed. The larger the tumor and the more bone that is removed, the more risk there is for loss of function or nerve damage. If surgery is the chosen treatment, complete tumor removal means better long-term outcomes. You and your care team will carefully consider the risks and benefits of surgery.

Depending on the complexity of the surgery, the procedure takes at least six hours and sometimes may need to be performed over the course of two days. Multiple surgeons may be involved, including orthopaedic, brain, head and neck, spine, general, and plastic surgeons. You will need to stay in the hospital for up to a week.

For skull base chordomas, surgeons may be able to use minimally invasive techniques through the nasal passages to reach the tumor. In these cases, you will have no visible scarring, a shorter hospital stay, and a quicker recovery.

Radiation Therapy
Duke radiation oncologists may recommend targeted courses of radiation therapy before surgery to shrink the tumor or after surgery to kill any remaining cancer cells. All radiation therapy is provided on an outpatient basis, so no hospital stay is required.

Follow-Up
Chordomas have about a 30% to 50% chance of recurrence. And, because the cancer is so slow-growing, it can metastasize years after successful treatment. We follow a strict surveillance schedule to catch any regrowth as soon as possible. For the first two years, you’ll be asked to return regularly for follow-up appointments and imaging scans. After that, appointments will space out incrementally over the next eight years.

Patient Navigation
Our nurse coordinators help you navigate your chordoma evaluation and treatment from start to finish. By gathering imaging and biopsy results, coordinating appointments, scheduling surgery, and answering any questions along the way, we help shepherd you through the process step-by-step.

Team of Experts 
Our team of orthopaedic surgical oncologists, skull base tumor experts, spine surgeons, and others work together to design a unique treatment plan, with your best interests in mind. Our radiologists specialize in bone tumors, and our pathologists are experts in distinguishing between chordomas and similar tumors. We also work with a range of other specialists who can address related issues.

Computer-Navigated Surgery
Our operating rooms are equipped with intraoperative CT and MRI imaging technology that helps us carefully plan for surgery and precisely remove your tumor. Deeply rooted bone sarcomas -- rare tumors that affect bone, muscle, fat, and other connective tissues -- can be challenging to remove completely, especially while trying to separate these tumors from important nerves and blood vessels that must remain unharmed. This technology allows our surgeons to identify your cancer and operate under the guidance of high-resolution, 3D imaging.

Comprehensive, High-Quality Cancer Care
As a National Cancer Institute-designated Comprehensive Cancer Center, we offer a level of expertise found only in the top cancer centers across the country. We are also part of the Sarcoma Alliance for Research through Collaboration, a group of leading sarcoma centers in the U.S. dedicated to improving the care of sarcoma patients through research and clinical trials.