Health Library
Chordoma in Children

What is Chordoma?

Chordoma is a slow-growing cancer of the spine. Chordoma tumors are commonly found at the skull base. The skull base is where the skull meets the neck. A chordoma in this location is called clival chordoma.

Chordoma tumors are rare, especially in younger children. As chordoma tumors grow, they can change the size or shape of nearby bones.

Chordoma Causes

There are no known causes of chordoma. Chordoma tumors form from cells left over after the spine develops.

Though chordoma cells develop early in life, chordoma tumors grow slowly. It can take years before symptoms develop.

Chordoma Symptoms

Chordoma tumors can occur anywhere along the spine so symptoms can vary. Chordoma symptoms also vary based on tumor size. People with a chordoma may have one or more of the following:

  • Difficulty swallowing
  • Eye movements and vision changes
  • Headache
  • Hearing problems
  • Muscle weakness or tremors
  • Neck pain
  • Snoring
  • Trouble with balance or coordination

Chordoma Diagnosis

Diagnosing chordoma involves a physical exam and imaging tests. Imaging tests used to diagnose chordoma include magnetic resonance imaging (MRI) and computed tomography (CT).

Doctors may also perform a biopsy to diagnose chordoma. A biopsy involves removing tissue from the tumor. Specialized doctors, called pathologists, then analyze the tissue to look for chordoma cells.

Chordoma Treatment

Chordoma treatment requires a multidisciplinary team of doctors and surgeons. Specialists in chordoma care include:

  • Cancer doctors
  • Ear, nose and throat doctors
  • Neurosurgeons
  • Plastic surgeons

Chordoma surgery is used to remove chordoma tumors. Surgeons use surgical resection to remove as much of the chordoma tumor as possible.

After surgery, some patients benefit from radiation therapy. Proton therapy is the best form of radiation therapy after chordoma surgery. It’s available at select hospitals and medical centers nationwide, including Cincinnati Children’s.

Proton therapy is a painless and precise form of radiation therapy. Unlike traditional radiation therapy, proton therapy is given by a very thin beam. Such targeted therapy protects nearby healthy tissue and organs. Protecting nearby tissue and organs reduces treatment side effects and long-term complications

Cincinnati Children’s Proton Therapy Center is one of just two proton centers worldwide owned by a children’s hospital.

Chordoma Prognosis

Chordoma affects each person differently. A person’s prognosis (long-term health) after a chordoma diagnosis will depend on:

  • Tumor location
  • If cancer has spread
  • How much of the tumor can be removed during surgery

Last Updated 12/2022

Reviewed By Smruti Patel, MD

Who treats this.

The Pituitary and Skull Base Surgery Center can help your child.