A chordoma is a rare tumour of notochordal cells. The majority occur in the spheno-occipital - clivus - and sacro- coccygeal areas, although they may arise anywhere within the spinal column between these two sites.
They are more common in males that females and usually present during the fourth decade of life. Growth begins in the midline but may expand asymmetrically. They are locally invasive and rarely metastasise.
Cranial chordomas often expand into the intracranial cavity where they present with multiple cranial nerve palsies ranging from ophthalmoparesis to bulbar effects. Chronic pain may develop from bone erosion.
Skull x-ray usually reveals a soft tissue mass with an osteolytic lesion in the skull base. This is confirmed by CT.
In the skull base, diagnosis should exclude meningioma, neurofibroma, carotid body tumour, carcinoma of the nasopharynx and Wegener's granulomatosis.
Treatment is by surgical excision when possible, although total removal is difficult. Alternatives are debulking operations and radiotherapy, although the latter seems to be relatively ineffective.
Most patients die within 10 years of presentation.
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