title: Recent advances in understanding and managing chordomas.
abstract: 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.
authors.affiliation: Department of Neurosurgery, University of Texas Southwestern Medical Center, Dallas, Texas, 75390, USA.
authors.forename: Carl
authors.initials: C
authors.lastname: Youssef
authors.affiliation: Department of Neurosurgery, University of Texas Southwestern Medical Center, Dallas, Texas, 75390, USA.
authors.forename: Salah G
authors.initials: SG
authors.lastname: Aoun
authors.affiliation: Department of Neurosurgery, University of Texas Southwestern Medical Center, Dallas, Texas, 75390, USA.
authors.forename: Jessica R
authors.initials: JR
authors.lastname: Moreno
authors.affiliation: Department of Neurosurgery, University of Texas Southwestern Medical Center, Dallas, Texas, 75390, USA.
authors.forename: Carlos A
authors.initials: CA
authors.lastname: Bagley
keywords: chordoma; chordomas; focused particle beam radiation; primary bone tumors; primary bone tumours; targeted chemotherapy