Background. Chordomas are uncommon primary malignant tumors of bone th
at typically occur in the axial skeleton including the sacrum, vertebr
ae, and skull base. The base of skull tumors usually are not amenable
to complete surgical resection, and most require postoperative radioth
erapy. The natural history of skull base chordoma is typified by slow
locally invasive tumor progression and eventual death, although few pa
rameters are known that allow stratification of patients into prognost
ic groups. Methods. Sixty-two patients with skull base chordomas treat
ed at the Massachusetts General Hospital by proton beam irradiation th
erapy with at least 2 years of follow-up information were reviewed in
an attempt to identify clinical and pathologic parameters that predict
ed outcome. Results. Female sex, tumor necrosis in preradiation treatm
ent biopsy, and tumor volume in excess of 70 ml were each independent
predictors of shortened overall survival after radiation therapy for s
kull base chordoma. Conclusions. Stratification of patients with skull
base chordoma into poor and good outcome groups can be performed usin
g the three parameters identified in our study. In addition, the strik
ing difference in survival between the sexes suggests that further inv
estigations of these tumors should include determination of their horm
onal receptor status and consideration of hormonal manipulation in the
ir management.