Tumour Group: Non-Malignant Brain Tumours
WHO Grade: The World Health Organization does not assign a grade to chordomas.
Prevalence/Incidence:  n/a
Typical Age Range: Most common in younger and middle-aged adults.

Description of Tumour
Treatment / Standard of Care

Description of Tumour

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 chordomas will be shown.

Expand for Prognosis Information

For brain tumour patients, a prognosis depends on several factors, which can include age and other health issues, the size of the tumour, its molecular profile, the type of tumour, how much can be removed and its response to treatments such as chemotherapy and radiation therapy.

Chordomas that are considered Grade I tend to have the most favourable survival rates compared to other higher grade brain tumours. Maximal safe removal, often combined with focused radiation therapy, can confer long-term survival in many patients. However lifelong surveillance of a chordoma is often required because recurrences are possible even years after initial diagnosis and treatment.


Adult Brain Tumour Handbook 6th Edition. Brain Tumour Foundation of Canada. 2012.

"Chordoma Foundation." Chordoma Foundation. N.p., n.d. Web. 09 Aug. 2013.

DiMaio, Salvatore, Dr. "Converging Paths to Progress for Skull Base Chordoma: Review of Current Therapy and Future Molecular Targets Di Maio S, Kong E, Yip S, Rostomily R - Surg Neurol Int." Converging Paths to Progress for Skull Base Chordoma: Review of Current Therapy and Future Molecular Targets Di Maio S, Kong E, Yip S, Rostomily R - Surg Neurol Int. N.p., n.d. Web. 09 Sept. 2013.

Louis, David N. WHO Classification of Tumours of the Central Nervous System. Lyon: International Agency for Research on Cancer, 2007. Print

Image credited to Hassan S, Abdullah JM, Wan Din SJ, Idris Z [CC-BY-2.0 (], via Wikimedia Commons


You can also download this information as a Information Sheet on Chordoma (pdf).


Candace's story with a ChordomaFor the past six years, Candace Graham’s life has been full of ups and downs – a “real rollercoaster,” she says. But still, this 32-year-old doesn’t let her circumstances set the tone for her life. Candace is a brain cancer survivor and this is her incredible story.




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