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目的探讨脊索样脑膜瘤的临床病理特征、诊断与鉴别诊断。方法观察小脑及鞍区的2例脊索样脑膜瘤的临床表现、组织学形态及免疫组化特征并复习相关文献。结果 2例患者均为成人,年龄47岁和68岁,肿瘤分别位于小脑和鞍区,因头痛或视力下降伴眼睑下垂就诊。镜下见黏液样基质中有呈分叶状或条索状排列的肿瘤细胞,细胞质淡染或嗜酸性,部分细胞呈液滴状,分布于黏液样基质中,类似脊索瘤样细胞;肿瘤局灶可见典型上皮型脑膜瘤区域。免疫组化:vimentin、EMA及D2-40(+),CKpan、S-100和GFAP(-)。结论脊索样脑膜瘤是一种罕见类型的脑膜瘤,发生在小脑及鞍区尤为少见,需结合临床病理、免疫表型明确诊断。肿瘤易复发,WHO分级Ⅱ级。
Objective To investigate the clinicopathological features, diagnosis and differential diagnosis of chorda-like meningioma. Methods The clinical manifestations, histological features and immunohistochemical features of 2 cases of cerebellomedullary meningioma in the cerebellum and the sellar area were observed and reviewed. Results Both patients were adults, aged 47 and 68 years old. The tumors were located in the cerebellum and the saddle area, respectively. The patients were diagnosed with headache or decreased visual acuity with ptosis. Microscopically seen mucus-like matrix in lobular or cord-like arrangement of tumor cells, light cytoplasm or eosinophilic, some cells were droplets, distributed in the mucoid matrix, similar to chordoma-like cells; tumor Board Typical lesions seen epithelial meningioma area. Immunohistochemistry: vimentin, EMA and D2-40 (+), CKpan, S-100 and GFAP (-). Conclusions Chordoid meningioma is a rare type of meningiomas that is particularly rare in the cerebellum and the sella. It needs to be clinically and pathologically characterized by a clear diagnosis of the immunophenotype. Tumor recurrence, WHO grade Ⅱ grade.