Combined endoscopic endonasal and posterior cervical approach to a clival chordoma

Summary

Chordomas are neoplasms of bone arising from the embryonic remnants of the notochord and comprising approximately 1% to 4% of all primary malignant bone tumours.¹ Of 400 cases recorded by the National Cancer Institute in the USA from 1973 to 1995, the distribution within the axial skeleton was reported as 32% at cranial sites, 32.8% spinal and 29.2% sacral.² The peak incidence of chordomas is in the fourth decade of life and it affects twice as many males as females.

Although slow-growing and rarely metastasising, clival chordomas have a poor prognosis due to their anatomical position, locally destructive capacity and late presentation when the lesion is large. Pain is the cardinal complaint with neurological deficits, commonly cranial nerve palsies, dependent on tumour location.¹ Median survival at 5 years is approximately 70%.¹ Surgery is widely considered the fundamental procedure in managing chordomas, whether supplemented by radiotherapy or not.³