What Is Chordoma?

Chordoma is a cancerous tumor that typically forms along the spine, most often at the base of the skull or in the lower spine. This type of tumor often grows slowly but tends to invade tissue surrounding the initial tumor and can often recur after surgery, making it difficult to treat. Chordoma occurs in the lower spine in adults over 50 and in the base of the skull in young adults and adolescents, although it can develop at any age. The disease is also rare, occurring in less than one out of every one million people yearly.

What Causes Chordoma?

The exact cause of chordoma is unclear. It arises from tissue from the notochord, which is a remnant structure from embryologic development that helps form the vertebrae. Some medical experts suspect that specific acquired genetic abnormalities have been linked to the T gene, which creates the protein brachyury, which is also expressed in notochordal tissue. In very rare instances, chordoma gets diagnosed in members of the same family.

Chordoma Cancer Symptoms

Due to the tumor’s tendency to grow slowly, chordoma symptoms don’t always surface right away. The symptoms of chordoma vary based on their size and location. An individual with a tumor on the base of the skull, called clival chordoma, typically experiences different symptoms than someone who develops chordoma in the lower back or sacral area.

Chordoma symptoms at the skull base usually include:

• Headache
• Difficulty swallowing
• Vision changes
• Dizziness or balance issues

Chordoma symptoms at the tailbone may consist of:

• Lower back pain
• Pain that runs down the leg (sciatica)
• Numbness or weakness in the lower extremities
• Bowel or bladder issues

Chordoma Diagnosis

Diagnosing chordoma usually involves various tests and consultations with neurologists, oncologists, surgeons, and other healthcare specialists.

Neurological Exam

Your physician will typically start by reviewing your medical history and performing a physical exam. They’ll check your balance, reflexes, muscle strength, and sensation.

Magnetic Resonance Imaging (MRI)

An MRI is an effective method of diagnosing chordoma. This imaging scan uses magnets and radio waves to give your physician clear, detailed images of the soft tissues and nerves surrounding the structures of your neck or spine. MRIs can detect abnormalities or compression of the spinal cord and can help determine the size and location of the tumor.

Computed Tomography (CT) Scan

Your team may also perform a CT scan in conjunction with an MRI. This technique is useful in assessing bone structures.

Needle Biopsy

Your physician extracts tissue and cell samples using a needle to confirm a chordoma diagnosis.

Chordoma Treatment

As chordoma grows or spreads to other areas, it can put pressure on the nerves in the spine or brain. Treatment may be necessary.

En Bloc Resection Surgery

This procedure involves removing the entire tumor without damaging surrounding structures, reducing the risk of the cancer coming back. Complete removal may not be possible, depending on the size or location of the tumor.

Radiation Therapy

You may have radiation therapy before and/or after surgery to kill any remaining cancer cells and lower the risk of the tumor recurring. Proton beam therapy may be used to deliver high radiation doses while avoiding damage to surrounding tissue.

Transoral Robotic Surgery (TORS)

If you have symptoms of clival chordoma (a tumor on the base of your skull), your surgeon may use this minimally invasive technique. TORS is an option for tumors in areas that are difficult to access through traditional surgery.

Chordoma Treatment Program

The multidisciplinary team at the Mass General Brigham Cancer Institute’s Center for Sarcoma and Connective Tissue Oncology provides patients with expert chordoma care. Learn more about the chordoma treatment program or request an appointment today.

Chordoma Prognosis

The prognosis for chordoma often depends on the size and location of the tumor, if it has spread to other areas of the body, and how well you respond to treatment. Your team will be able to answer any questions related to your specific case.

FAQs About Chordoma

Is Chordoma Curable?

If the tumor is detected early on and can be removed completely, it’s possible it can be cured.

Is Chordoma Benign or Malignant?

Chordoma tumors are considered malignant.

Who Is Most Likely to Get Chordoma?

Chordoma of the lower spine typically occurs in people 50 to 80 years old, and chordoma of the skull base can occur in younger adults or adolescents. Biological males are more predisposed to the disease than females.