Skull base chordomas: clinical features, prognostic factors, and therapeutics.

Chordomas of the skull base are one of the rarest intracranial malignancies that arise from ectopic remnants of embryonal notochod. The proximity of many chordomas to neurovascular structures makes gross total resection difficult, and the tendency for recurrence leads to the routine use of adjuvant postoperative radiation. Several surgical approaches are used ranging from extensive craniotomies to minimally invasive endonasal endoscopic approaches. In this review, the histopathology and epidemiology, imaging characteristics, surgical approaches, adjuvant therapies, prognostic factors, and molecular biology of chordomas are described.