title: The molecular aspects of chordoma.
abstract: Chordomas are one of the rarest bone tumors, and they originate from remnants of embryonic notochord along the spine, more frequently at the skull base and sacrum. Although they are relatively slow growing and low grade, chordomas are highly recurrent, aggressive, locally invasive, and prone to metastasize to the lungs, bone, and the liver. Chordomas highly and generally show a dual epithelial-mesenchymal differentiation. These tumors resist chemotherapy and radiotherapy; therefore, radical surgery and high-dose radiation are the most used treatments, although there is no standard way to treat the disease. The molecular biology process behind the initiation and progression of a chordoma needs to be revealed for a better understanding of the disease and to develop more effective therapies. Efforts to discover the mysteries of these molecular aspects have delineated several molecular and genetic alterations in this tumor. Here, we review and describe the emerging insights into the molecular landscape of chordomas.
authors.affiliation: Department of Genetics and Bioengineering, Faculty of Engineering and Architecture, Yeditepe University, Istanbul, Turkey.|Department of Medical Genetics, Yeditepe University Medical School and Yeditepe University Hospital, Istanbul, Turkey.
authors.forename: Sukru
authors.initials: S
authors.lastname: Gulluoglu
authors.affiliation: Department of Genetics and Bioengineering, Faculty of Engineering and Architecture, Yeditepe University, Istanbul, Turkey.
authors.forename: Ozlem
authors.initials: O
authors.lastname: Turksoy
authors.affiliation: Department of Medical Genetics, Yeditepe University Medical School and Yeditepe University Hospital, Istanbul, Turkey.
authors.forename: Aysegul
authors.initials: A
authors.lastname: Kuskucu
authors.affiliation: Department of Neurosurgery, Yeditepe University Medical School and Yeditepe University Hospital, Istanbul, Turkey.
authors.forename: Ugur
authors.initials: U
authors.lastname: Ture
authors.affiliation: Department of Medical Genetics, Yeditepe University Medical School and Yeditepe University Hospital, Istanbul, Turkey. ofbayrak@yeditepe.edu.tr.
authors.forename: Omer Faruk
authors.initials: OF
authors.lastname: Bayrak
mesh_terms: D016723:Bone Remodeling / Q000235:genetics*; D002817:Chordoma / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology*; D018450:Disease Progression; D006801:Humans; D019291:Skull Base / Q000378:metabolism* / Q000473:pathology; D019292:Skull Base Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000378:metabolism; D013131:Spine / Q000378:metabolism* / Q000473:pathology
keywords: Chordoma; Gene expression; Molecular pathology; Molecular pathways