中枢性原始神经外胚层肿瘤16例临床病理分析

目的]探讨中枢性原始神经外胚层肿瘤(PNETs)的病理诊断、鉴别诊断及意义。方法]采用光镜、免疫组织化学、特染及电镜对16例PNETs病例进行观察。结果]肿瘤细胞排列成巢状、团块状或散在分布,细胞呈小圆形,大小一致,有菊形团形成;免疫组化显示有两项以上神经源性抗体呈阳性表达,以CD99最为显著;特殊染色显示瘤组织间网状纤维明显减少,部分瘤细胞胞浆内有糖原颗粒;电镜下部分瘤细胞胞浆内有神经内分泌颗粒。结论]中枢性原始神经外胚层肿瘤归于神经上皮组织的胚胎性肿瘤,属高度恶性,诊断时须与髓母细胞瘤等相鉴别。

Primitive Neuroectodermal Tumors of The Central Nervous System:A Clinicopathological Study of 16 Cases

To in vestigate the pathological diagnosis and differential diagnosis of primitive neu roec-todermal tumors of the central nervous system.Morphologic studies and peculiar staining,immuno-histochemical techniques and electron-microscope analyses were performed on16cases of the primitive neu-roectodermal tumors of the central nervous system.The tumor cells were usually small,round and monotonous and appeared as nests,cords and diffuse distribution and Homer Wrigh t rosettes.The immuno-histochemistry assay showed positive expression of more t han two kinds of the neurogenous antibody,espe-cially CD99markedly strong expre ssion.Specific staining showed that reticular fibers markedly decreased and some tumor cells had cytoplasmic glycogen granules.At the ultrastructural level some tumor cells had nervous-endocrinous granules in cytoplasm.Conclusions]The pri mitive neuroectodermal tumors of the cen-tral nervous system is a highly malign ant embryonal neuroepithelial neoplasm.It should be differentiated from medullob lastoma.