脊索瘤来源于脊索胚胎残余组织产生的原发性骨性肿瘤,是一种少见的慢性进展的低度恶性肿瘤,占全部原发恶性肿瘤的3%～4%,居原发性恶性骨肿瘤第4位。有约1/3起源于颅底部,其余可起源于脊柱,骶尾部或中线以外的位置, Chordoma originates from the primary bone tumor produced by the residual tissue of the spinal cord embryo. It is a rare chronic low-grade malignant tumor accounting for 3% ~ 4% of the total primary malignant tumors, ranking as the primary malignant bone tumor 4th place. About 1/3 originated in the skull base, the rest can originate in the spine, sacrococcygeal or midline position,