Chordoma Tumors

Overview
Symptoms & Diagnosis
Treatment

What is Chordoma?

Chordomas are a type of primary bone cancer usually found in the skull or spine. These slow-growing tumors are rare, accounting for only 1 percent of central nervous system cancers.

Chordomas develop from remnants of notochord, which precedes the spinal column during embryonic development.

While chordomas may be found at any site along the axial skeleton, they are most frequently found at the base of the spine (sacrum), tailbone (coccyx), and base of the skull (clivus).

Chordoma cancers rarely spread (metastasize), but they are aggressive locally. They can affect adjacent organs, tissues, and bone.

Symptoms of chordomas can include back pain, numbness, headaches, and vision problems.

This type of tumor has a high recurrence rate. Chordomas are most commonly found in adults between the ages of 40 and 70.

Treatments for chordoma include surgery, chemotherapy, and radiation therapy.

At UPMC, the preferred surgical treatment for chordomas of the skull base is the Endoscopic Endonasal Approach (EEA). This innovative, minimally invasive technique uses the nose and nasal cavities as natural corridors to access hard-to-reach or previously inoperable tumors. Benefits of EEA include:

No incisions to heal
No disfigurement
Faster recovery time

Diagnosing Chordoma

Early diagnosis of chordoma may prevent serious complications.

Your doctor will:

Ask you about your symptoms
Perform a physical exam
Order imaging tests

Symptoms of chordoma in the skull base

Skull base chordoma symptoms may include:

Headache
Double vision
Facial pain
Changes in hearing
Difficulty swallowing

Imaging and radiology tests for diagnosing chordoma tumors

In addition to a physical exam, your doctor may request imaging tests, such as MRI or CT scans.

Chordoma Treatments

Three standard treatments for skull base chordoma are surgery, chemotherapy, and radiation therapy.

The neurosurgical team at UPMC may recommend one or a combination of these treatments. The high recurrence rate of these tumors requires frequent follow-up and monitoring.

Chordoma surgery

Chordoma surgery is the main type of treatment for bone cancers. The type of surgical procedure depends on the size and location of the tumor.

Chordomas of the skull base may be approached directly using the Endoscopic Endonasal Approach (EEA).This state-of-the-art, minimally invasive treatment approach allows surgeons to access the tumor through the natural corridor of the nose, without making an open incision. Surgeons then remove the chordoma through the nose and nasal cavities.

EEA offers the benefits of no incisions to heal, no disfigurement, and a faster recovery time.

If you need complementary treatments, such as radiation, those therapies can begin soon after EEA surgery.

Radiation therapy for chordomas

Your doctor may deliver radiation therapy:

Externally, by directing radiation at the chordoma from an outside source.
Internally, by placing radioactive material directly in the body near the chordoma tumor.
Using stereotactic radiosurgery, by sending a concentrated dose of radiation directly to tumor.

The preferred type of radiation treatment for chordomas is proton beam radiation.