Cranial base chordoma, a rare, usually slow-growing tumour of bone tho
ught: to arise in notochord remnants, presents itself when its growth
in the spheno-occiput results in cranial nerve compression or an obvio
us nasopharyngeal mass. The present review of 11 cases of cranial base
chordoma encountered at three McGill University teaching hospitals fr
om 1979 until 1992 examines presentation, radiologic findings, patholo
gy, treatment, and outcome. A literature review surveys chordoma with
regard to epidemiology, diagnosis, efficacy of medical and surgical mo
dalities on tumour control, and survival. Discussion focuses on curren
t therapeutic options relative to histopathologic characteristics of c
hordoma, and whether or not these may be used to predict course of dis
ease and outcome.