Converging paths to progress for skull base chordoma: Review of current therapy and future molecular targets

BACKGROUND: Chordomas of the skull base are rare locally aggressive neoplasms with a predilection for encapsulating critical neurovascular structures, bony destruction and irregular growth patterns, and from which patients succumb to recurrence and treatment failures. METHODS: A review of the medical literature is performed, using standard search engines and identifying articles related to skull base chordomas, surgery, radiation therapy, chemotherapy, molecular genetics, and prospective trials. RESULTS: A synthesis of the literature is presented, including sections on pathology, treatment, molecular genetics, challenges, and future directions. CONCLUSION: Beyond an understanding of the current treatment paradigms for skull base chordomas, the reader gains insight into the collaborative approach applied to orphan diseases, of which chordomas is a prime exemplar.