目的探讨脊索样胶质瘤的临床病理学特征、免疫表型、诊断及鉴别诊断。方法对6例脊索样胶质瘤患者的临床、影像学及组织病理学资料进行回顾分析。结果 6例患者中女性5例,男性1例,发病年龄23~58岁,平均年龄40.7岁。临床主要症状为记忆力减退、头痛、呕吐和嗜睡等,5例女性患者伴有不同程度的月经失调。组织学特征为黏液基质中可见团状或条索状的GFAP阳性的上皮样瘤细胞,伴有多少不等的淋巴浆细胞浸润。免疫组化:瘤细胞GFAP、vimentin、EMA和S-100均(+);且黏液基质PAS(+)。结论脊索样胶质瘤位于第三脑室及周围区,是中枢神经系统独特的罕见肿瘤,结合组织学形态、免疫组化及组织化学染色可明确诊断。 Objective To investigate the clinicopathological features, immunophenotype, diagnosis and differential diagnosis of chordoid glioma. Methods The clinical, radiological and histopathological data of 6 patients with chordoid glioma were retrospectively analyzed. Results Among the 6 patients, 5 were women and 1 was male, with a mean age of 40.7 years and 23- 58 years of age. The main clinical symptoms of memory loss, headache, vomiting and drowsiness, 5 cases of female patients with varying degrees of menstrual disorders. Histological features of the mucus matrix visible clumps or cords of GFAP-positive epithelioid tumor cells, with varying degrees of lymphoplasmacytic infiltration. Immunohistochemistry: tumor cells GFAP, vimentin, EMA and S-100 (+); and mucus matrix PAS (+). Conclusion Chordoid glioma is located in the third ventricle and the surrounding area. It is a rare and unique tumor of the central nervous system. Combined with histological morphology, immunohistochemistry and histochemical staining can confirm the diagnosis.