目的：探讨脊索样脑膜瘤(chordoid meningioma，CM)的临床、影像及病理特征。方法：回顾性分 析15例CM的临床病理特征并复习相关文献。结果：15例CM中女性占多数(女：男=11:4)，年龄 15~71岁，平均47.4岁。主要发生部位为额顶叶凸面、蝶骨嵴。以头痛、视力模糊、肢体乏力、 面部感觉异常和言语含糊等为主要表现就诊。影像学主要表现为典型脑膜瘤特征，如界限清楚、 脑膜尾征以及粘液成分引起的高低混杂信号等。组织病理学除表现典型脑膜瘤外，见不同比例 (20%~100%)的脊索样成分，即嗜碱性的黏液背景中肿瘤细胞呈条索状、筛网状、假菊型团样或巢 团状排列，细胞呈上皮样或胖梭形细胞，胞浆丰富嗜酸性，部分含脂滴样空泡。免疫组织化学及 组织化学染色显示Vimentin、EMA、PR及PAS阳性，S-100、CK和D2-40局灶阳性，GFAP阴性， Ki-67增殖指数1%~10%，平均3%。13例行全切手术，其中1例术后辅助放疗，另外2例行次全切 除。随访时间1~120月不等，结果显示2例肿物次全切除者术后复发，其余均无瘤生存。结论：CM 好发于中老年女性。临床症状多样。影像学对具有典型影像特征的脑膜瘤有提示作用，反之易误 诊，诊断及鉴别诊断依赖镜下形态和免疫组织化学染色等。肿瘤切除程度与预后密切相关，对于 次全切除患者建议术后进一步辅助放疗。
Chordoid meningioma: a report of 15 cases
Objective: To explore the clinical, imaging and pathological features of chordoid meningioma (CM). Methods: Retrospectively analysis the clinicopathologic features of 15 CM and review related literatures. Results: There was obvious female preponderance with male to female ratio of 4:11, the average age is 47.4 (15-71) years old. Mostly happens in the convex of frontal and parietal lobe, sphenoid ridge. Clinical symptoms including headache, blurred vision, weakness of limbs, facial paresthesia and slurred speech, and so on. Imaging mainly manifests the typical features of meningiomas, such as clear boundaries, dural tail sign and high-low mixed signs caused by mucus. Microscopically, in addition to the typical meningioma, there were different proportion of chordoid components (20%~100%), that was, tumor cells arrange to cords, mesh-like, false daisy-type groups or nest pattern in the background of basophilic mucus. Cells show epithelioid or fat spindle, with an abundant of eosinophilic cytoplasm. Some contain lipid droplet-like vacuoles. Immunohistochemically and histochemically, vimentin, EMA, PR and PAS were positive, and S-100, CK and D2-40 are focal positive, and GFAP was negative, proliferation index of Ki-67 with an average of 3% (1%~10%). A total of 13 cases were performed total removal, including one case which given radiotherapy after operation, and two cases for subtotal removal. Follow-up time ranges from 1-120 months. Two cases recurred after operation, and all the rest remain free of chordoid meningiomas. Conclusion: Chordoid meningiomas occur in middle-aged women, with diverse clinical symptoms. Imaging are suggestive when meningioma has typical features, whereas easily misdiagnosis. Diagnosis and differential diagnosis depends on the morphology and immunohistochemistry. The prognosis of CM is closely related to the degree of surgical resection. Patients with subtotally resect are suggested to accept postoperative radiotherapy.