Health Encyclopedia

Skull Base Chordoma

A chordoma is a form of bone cancer that can occur anywhere along the length of the spine, from the base of the skull to the lower back. When it occurs at the base of the skull, it is known as skull base chordoma. Because of its location in the base of the skull, it commonly affects vital structures, such as the nerves that control movement of the face, eyes, and swallowing. 

Facts about skull base chordoma

Chordomas are rare cancers. Anyone can develop a chordoma at any age,but they are more common in older adults.

Types of skull base chordoma

Skull base chordomas are divided into 3 types:

  • Conventional. This is the most common type of chordoma and is sometimes called a classic chordoma.

  • Chondroid. This type of chordoma has about the same outlook as conventional chordomas.

  • Dedifferentiated. The dedifferentiated form of chordoma is more aggressive and grows more quickly than the other types. 


If you have a skull base chordoma, the most common symptoms that you will experience are related to pain or changes in nerve function. These can include:

  • Headache

  • Neck pain

  • Double vision

  • Numbness in the face

  • Paralysis of the facial muscles

  • Speech changes

  • Swallowing problems


Techniques typically used to diagnose cancer will be used to identify a skull base chordoma. These may include various scans, such as X-rays, CT scans or MRI, a blood test, or a biopsy. Testing may be done by a specialist, such as a neurologist or a neurosurgeon.

One common misdiagnosis that can occur with a chordoma is confusing it with a chondrosarcoma, another type of bone tumor. Both types of tumors can occur in similar locations in the body, and they look similar as well. Chondrosarcomas tend to respond better to radiation and have a better outlook.


The primary treatment for a skull base chordoma is surgery to remove as much of the tumor as possible. This is often an aggressive procedure that can lead to some side effects, but it offers the best chance for long-term survival. You and your health care team should weigh the risks and benefits of the procedure before the surgery.

Once the surgery is complete, an aggressive course of radiation therapy is used to kill the surrounding tumor cells. Proton beam radiation is often used because it can focus on the needed area without exposing any critical organs and structures around it to radiation. Proton beam radiation offers the advantage of increasing the tumor dose while minimizing the dose to the adjacent normal tissue. Other forms of radiation are sometimes used as well. 

Chemotherapy is typically not effective in treating a skull base chordoma. Currently, no drugs are approved for treating chordomas, but some have shown effectiveness in clinical trials.


Skull base chordomas are rare disorders, and it is not known how to prevent them.

Managing skull base chordomas

A skull base chordoma is a malignant and potentially life-threatening tumor. People need to be prepared for a difficult fight against the illness, one that will involve challenging treatments, such as surgery and radiation therapy.

Outcomes from chordoma treatment have improved in recent decades. Still, it's best to address potential end-of-life issues as part of a skull base chordoma management plan. 

Medical Reviewers:

  • Alteri, Rick, MD
  • Cunningham, Louise, RN