Recent advances in understanding and managing chordomas

Chordomas are rare primary bone tumors arising from embryonic remnants of the notochord. They are slow-growing, locally aggressive, and destructive and typically involve the axial skeleton. Genetic studies have identified several mutations implicated in the pathogenesis of these tumors. Treatment poses a challenge given their insidious progression, degree of local invasion at presentation, and high recurrence rate. They tend to respond poorly to conventional chemotherapy and radiation. This makes radical resection the mainstay of their treatment. Recent advances in targeted chemotherapy and focused particle beam radiation, however, have improved the management and prognosis of these tumors. Carlos A. Bagley ( ) Corresponding author: [email protected] Youssef C, Aoun SG, Moreno JR and Bagley CA. How to cite this article: Recent advances in understanding and managing chordomas 2016, (F1000 Faculty Rev):2902 (doi: ) [version 1; referees: 2 approved] F1000Research 5 10.12688/f1000research.9499.1 © 2016 Youssef C . This is an open access article distributed under the terms of the , which Copyright: et al Creative Commons Attribution Licence permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The author(s) declared that no grants were involved in supporting this work. Grant information: Competing interests: The authors declare that they have no competing interests. 22 Dec 2016, (F1000 Faculty Rev):2902 (doi: ) First published: 5 10.12688/f1000research.9499.1 Referee Status: