Skull base chordomas: overview of disease, management options, and outcome.

Cranial base chordomas are locally invasive tumors that, from a midline, clival location, extend in different directions and display various patterns of skull base invasion. Although histologically benign, their invasive nature makes true "oncological" resection virtually impossible to achieve in most cases, despite modern skull base surgical techniques. Moreover, because of the tumor's location and proximity to critical neural and vascular structures, surgery-related morbidity can be significant when an aggressive resection is undertaken. Cytoreductive surgery assumes a critical role in the management of these lesions. The choice of surgical approach and the extent of resection are dependent on several factors: location and extension of the tumor, the surgeon's philosophy and familiarity with a specific approach, and the patient's preexisting clinical status. Proton-beam radiotherapy seems to be effective as an adjunct to surgery in achieving local tumor control. The timing of radiation therapy, however, remains controversial. Gamma knife surgery has been proposed as an adjunctive therapy, but the limited experience and short follow-up periods do not permit formulation of meaningful conclusions at this time. Recurrences are common, although in a subset of patients prolonged disease-free survival is demonstrated.