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目的 :探讨原发于大脑和脊髓原始神经外胚叶肿瘤的临床病理特征、组织学发生及生物学行为。方法 :采用组织病理学、免疫组化标记和电镜观察对 11例原发于大脑及脊髓的原始神经外胚叶肿瘤进行研究。结果 :组织学特征为弥漫原始的小细胞 ,并形成Homer Wright菊形团和明显的纤维组织分隔成小叶。免疫组化标记 :NSE阳性 ,Syn阳性 ,部分瘤组织中散在GFAP阳性。电镜 :瘤细胞胞浆内可见致密核心的分泌颗粒。结论 :该肿瘤和小脑髓母细胞瘤的病理形态特征、标志物表达及生物学行为相同 ,同属原始神经外胚层肿瘤的范畴。
Objective : To investigate the clinicopathological characteristics, histogenesis and biological behavior of primitive neuroectodermal tumors originating from the brain and spinal cord. METHODS: Eleven cases of primitive neuroectodermal tumors originating from the brain and spinal cord were studied by histopathology, immunohistochemistry and electron microscopy. RESULTS: Histological features were diffused in the primitive minicells and formed Homer Wright chrysanthemums and distinct fibrous tissue separated into leaflets. Immunohistochemical markers: NSE positive, Syn positive, scattered GFAP positive in some tumor tissues. Electron microscopy: Secretory granules of dense cores can be seen in the cytoplasm of tumor cells. Conclusion : The pathological features, marker expression and biological behaviors of the tumor and cerebellar medulloblastoma are the same and belong to the category of primitive neuroectodermal tumors.