Chordoma: current concepts, management, and future directions.
Chordoma is a rare bone cancer that is aggressive, locally invasive, and has a poor prognosis. Chordomas are thought to arise from transformed remnants of notochord and have a predilection for the axial skeleton, with the most common sites being the sacrum, skull base, and spine. The gold standard treatment for chordomas of the mobile spine and sacrum is en-bloc excision with wide margins and postoperative external-beam radiation therapy. Treatment of clival chordomas is unique from other locations with an enhanced emphasis on preservation of neurological function, typified by a general paradigm of maximally safe cytoreductive surgery and advanced radiation delivery techniques. In this Review, we highlight current standards in diagnosis, clinical management, and molecular characterisation of chordomas, and discuss current research.
Department of Neurosurgery, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA. walcott.brian@mgh.harvard.edu
Brian P
BP
Walcott
Brian V
BV
Nahed
Ahmed
A
Mohyeldin
Jean-Valery
JV
Coumans
Kristopher T
KT
Kahle
Manuel J
MJ
Ferreira
D001859:Bone Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D002817:Chordoma / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D017321:Clinical Trials, Phase I as Topic; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D011379:Prognosis; D035583:Rare Diseases / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D017479:Receptors, Platelet-Derived Growth Factor / Q000378:metabolism; D013131:Spine / Q000473:pathology; D016896:Treatment Outcome
D017479:Receptors, Platelet-Derived Growth Factor