Chordomas of the craniospinal axis: Multimodality surgical, radiation and medical management strategies

Chordomas are neoplasms of the primitive notochord remnants and are characterized by slow growth kinetics, locally aggressive behavior and resistance to conventional therapeutic options. They are found primarily in the skull base or the sacral region, although they can occur anywhere in the craniospinal axis. If an oncologic surgical resection can be performed safely, patients derive the maximal benefit. Adjuvant radiotherapy has a proven benefit in both progression-free and overall survival. Chemotherapy plays a limited role and currently remains an option at tumor recurrence, although increasing knowledge of the molecular biology of chordomas may lead to targeted therapeutic strategies. In this review, the current multimodality treatment strategy for chordomas will be discussed and future directions will be highlighted.