脊索瘤是一种少见的、具有局部破坏性的肿瘤,起源于错置或残留的胚胎脊索组织。脊索瘤属低度恶性肿瘤,很少发生转移。颅底脊索瘤多发生在颅底蝶枕交界部,常见于斜坡、鞍区和中颅窝,颈静脉孔区亦可发生脊索瘤,但较为罕见。其自然病程表现为持续加重的颅底骨侵蚀以及邻近神经血管 Chordoma is a rare, locally destructive tumor that originates from mismatched or residual embryonic chordal tissue. Chordoma is a low-grade cancer, rarely happen metastasis. Skull base chordoma occurred in the skull at the junction of the pillow, common in slopes, saddle area and the middle cranial fossa, jugular foramen may also occur chordoma, but relatively rare. The natural course of the disease is manifested by the continued increase of skull base erosion and adjacent neurovascular