title: Chordomas: A Review.
abstract: Chordomas (CHs) are tumors with benign histopathology but exhibit aggressive clinical behavior with invasive and metastatic potential. CHs are challenging tumors to treat, and there are still many questions especially about their optimum treatment. Radical resection at first presentation should be applied on all CHs. Complementary proton therapy irrespective of the quality of resection is now routinely proposed even if its efficacy is not clearly demonstrated in case of incomplete resection. The future of CH treatment is certainly related to a better understanding of the molecular biology and oncogenesis of CHs and consequently to the development of efficient targeted chemotherapies.
authors.affiliation: Department of Neurosurgery, Hôpital LARIBOISIERE, Assistance Publique - Hôpitaux de Paris, Université Paris Diderot, 2 rue Ambroise Paré, Paris 75475, France.
authors.forename: Bernard
authors.initials: B
authors.lastname: George
authors.affiliation: Department of Neurosurgery, Hôpital LARIBOISIERE, Assistance Publique - Hôpitaux de Paris, Université Paris Diderot, 2 rue Ambroise Paré, Paris 75475, France.
authors.forename: Damien
authors.initials: D
authors.lastname: Bresson
authors.affiliation: ENT Department, Hôpital LARIBOISIERE, Assistance Publique - Hôpitaux de Paris, Université Paris Diderot, 2 rue Ambroise Paré, Paris 75475, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Herman
authors.affiliation: Department of Neurosurgery, Hôpital LARIBOISIERE, Assistance Publique - Hôpitaux de Paris, Université Paris Diderot, 2 rue Ambroise Paré, Paris 75475, France. Electronic address: sebastien.froelich@lrb.aphp.fr.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Froelich
mesh_terms: D001706:Biopsy; D002817:Chordoma / Q000175:diagnosis / Q000378:metabolism / Q000628:therapy*; D005326:Fetal Proteins / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D057605:Natural Orifice Endoscopic Surgery; D044583:Neuroendoscopy / Q000379:methods*; D061766:Proton Therapy; D018714:Radiotherapy, Adjuvant; D019292:Skull Base Neoplasms / Q000175:diagnosis / Q000378:metabolism / Q000628:therapy*; D020825:T-Box Domain Proteins / Q000378:metabolism; D000098908:Brachyury Protein
chemical_list: D005326:Fetal Proteins; D020825:T-Box Domain Proteins; D000098908:Brachyury Protein
keywords: Chordomas; Endonasal approach; Proton beam therapy