脊索样脑膜瘤是脑膜瘤的亚型之一,WHOⅡ级,临床少见,发病年龄略早于良性脑膜瘤,无明显男女性别差异,儿童患者可合并Castleman综合征。该病术前诊断困难,需要与良性脑膜瘤、脊索瘤、软骨肉瘤、脊索样胶质瘤相鉴别。脊索样脑膜瘤首选手术治疗,然而手术全切除困难,术后易复发,尤其是非全切除术后、脊索样成分较高和Ki-67指数高的患者,术后需辅助放射治疗及长期随访。 Chordate-like meningioma is one of the subtypes of meningioma, WHO Ⅱ grade, clinical rare, slightly older age than benign meningioma, no significant gender differences in children, children with Castleman syndrome may be combined. The preoperative diagnosis is difficult, need and meningioma, chordoma, chondrosarcoma, chordate glioma phase identification. Chordate-like meningioma surgery is the first choice, however, the total excision of the surgical difficulties, postoperative recurrence, especially after non-total resection, higher chordoid-like composition and Ki-67 index in patients with postoperative adjuvant radiotherapy and long-term follow-up.