大脑和脊髓原始神经外胚叶肿瘤临床病理学

A clinicopathological study of primitive neuroectodermal tumors of cerebrum and spinal cord: A report of 11 cases

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。

Purpose To investigate in clinicopathological features, histogenesis and biological behavior of primary primitive neuroectodermal tumors of cerebrum and spinal cord(PNTCSC). Methods Eleven cases of PNTCSC were studied with histologic and immunohistochemical staining and electron microscope. Results Histological features were Homer Wright rosetts formed by primitive small cells and leaflets seperated by fibrous tissue. Immunohistochemical reaction revealed positive staining for NSE and Syn, partially for GFAP. Ultrastructure showed that densie nuclear secretory granules in the tumor cells cytoplasm. Conclusion The tumor is similar to cerebellar medulloblastoma in pathological morphological features, specific marker expression and biological behavior, both belonging to the same category.