Intracranial chordomas are tumors arising from embryonic vestiges of the notochord (chorda dorsalis). Although these tumors remain a rare pathologic curiosity, they are of distinct clinical interest. Because of their usual position at the base of the brain, in the region of the sella turcica, they may simulate other tumors more commonly present in this region, such as chromophobic adenoma of the pituitary, craniopharyngioma, parasellar meningioma, dermoid cyst and carcinoma arising from the sphenoid sinus or in the nasopharynx with intracranial extension.
Chordoma was the name applied to these tumors by Ribbert,1 in 1894. The notochord, which forms the primitive axial support of all vertebrates, extends from head to tail in the midline (Arey2). In later stages the primitive backbone becomes enclosed by a stiffer axial skeleton in the bodies of the vertebrae and in the basilar plate of the skull. Eventually the notochord degenerates. In adult man
ZEITLIN H, LEVINSON SA. INTRACRANIAL CHORDOMA. Arch NeurPsych. 1941;45(6):984-991. doi:10.1001/archneurpsyc.1941.02280180096007