Skull Base Chordomas

Chordomas are rare tumors originating from the remnants of the primitive notochord. Their deep location and patterns of extension and the proximity of structures such as the brain stem, upper cervical spine, hypophysis, and cranial nerves make their treatment a challenge. Even if gross total removal has been achieved, the malignant histologic patterns, the presence of isolated islands of tumor into normal bone, the extension of the lesion through the skull base, and the deep and central location preclude an en bloc oncologic resection. During the past decade, several new surgical approaches and new modalities of radiation therapy have been developed for treating these lesions. In this review, the diagnosis and management of skull base chordomas are discussed, with emphasis on radical surgery treatment with radiation therapy with heavy particles. Despite the absence of malignant pathologic findings, the uncertain behavior of some lesions makes the management of skull base chordomas a “no-man's land.” A review of the current literature allows the conclusion that radical surgical removal associated with adjuvant radiation therapy with heavy particles is, up to now, the best way to treat chordomas of the skull base.