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Tumour Group:
Non-Malignant Brain Tumours
WHO Grade:
WHO does not assign a grade to chordomas.
Typical Age Range:
Most common in younger and middle-aged adults.

Tumour Characteristics

Chordomas are usually slow-growing, locally invasive tumours occurring at the base of the skull or at the end of the spine. They affect the adjacent cranial nerves and brainstem.

  • Originates from cells left over from early fetal development
  • Invades the bone and soft tissues, and sometimes involves the brain and cranial nerves
  • Can block the ventricles, causing hydrocephalus (water on the brain)
  • Can metastasize (spread) or recur


Common symptoms include, but are not necessarily limited to:

  • Double vision
  • Headaches

Treatment/Standard of Care

It is generally agreed that the optimal treatment for most skull-base chordomas is maximal surgical removal followed by focused radiation therapy. Skull-base chordomas are difficult tumours to remove, and it is important that patients are referred to neurosurgeons with particular expertise in skull base chordomas. Some chordomas may also be amenable to resection via endoscopic surgery through the nasal cavities.

There are many types of radiation therapy possible for chordomas, including proton beam, linac radiotherapy, IMRT, gamma knife, carbon ion and others. In rare cases, chemotherapy may also be recommended as part of the treatment plan.


A prognosis is an estimate of the likely progress of a disease after a diagnosis, based on an average patient group. Since every person is different, please take time to talk with your health care team about how this information applies to you.

By clicking on 'expand', a statistic on the prognosis for Chordoma will be shown.

Download this information as an Informative Sheet PDF on Chordoma

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