Chordomas make up about 5-10% of primary spinal cord tumours. They arise at several levels:

  • Base of skull: 25%, presenting with cranial nerve abnormalities and headache
  • Cervical spine: 15%, presenting with pain, radiculopathy or myelopathy
  • Thoracolumbar spine: 10%
  • Sacrococcygeal: 50%, presenting with lower back pain, sacral nerve abnormalities

The main differential diagnosis varies; in the base of skull it is chondrosarcoma.
Patients usually present with gradual onset of symptoms over years due to the slow growing nature of the lesion.

Imaging Appearance


Maximal surgical resection is ideal but gross total resection is rarely possible. Radiotherapy is typically recommended to improve local control (dose 55-70 Gy). 5 year survival is about 50%. Local control with individual modalities is < 20%; with combined treatment it approaches 40-50%.
In the USA, treatment with protons or stereotactic radiotherapy is becoming more popular.