形成菊形团的胶质神经元肿瘤临床病理观察

目的:探讨形成菊形团的胶质神经元肿瘤(RGNT)的临床病理学特征.方法:对1例发生于第四脑室形成菊形团的胶质神经元肿瘤的临床表现、影像学、组织形态学、免疫组化以及分子病理学等结果进行分析,并结合文献探讨其诊断与鉴别诊断要点.结果:组织学上表现为神经细胞和毛细胞样星形细胞并存,神经细胞较小、一致,围绕神经毡结构形成菊形团样结构或围绕血管形成假菊形团样结构.胶质成分形态学符合毛细胞星形细胞瘤.免疫组化显示:神经毡样基质呈Syn、NSE阳性,GFAP、CgA阴性.神经细胞核S-100、Neu-N阳性.胶质成分呈GFAP、S-100阳性,Ki-67<2%.结论:形成菊形团的胶质神经元肿瘤是混合性胶质神经元肿瘤中的少见类型,2007年列入第四版中枢神经系统WHO,是WHO I级的低级别胶质瘤.组织学上以胶质成分及围绕神经毡呈菊形团排列的神经细胞成分为主,免疫组化有鉴别诊断意义.

Clinicopathological features of Rosette-forming glioneuronal tumor

Objective:To explore the clinicopathologic characteristics of Rosette-forming glioneuronal tumor (RGNT).Methods:In this study,we presented one case of RGNT of the fourth ventricle.The clinical and imaging features,detailed histopathological,immunohistochemical and molecular pathological profile were discussed and the published literature was reviewed.Results:Histologically,two distinct regions characterised by a rosette-forming neurocytic component and an astrocytic predominant component can be examined.Rosenthal fibers and eosinophilic granular bodies were occasionally identified.Neurocytic rosettes were detected in the neurocytic component,and the center of rosettes showed positive immunostaining for synaptophysin and NSE,but negative for GFAP and CgA.The neuronal nuclei stained positive for Neu-N and S-100,and the glial components were positive for GFAP and S-100,with Ki-67 labeling index <2%.Conclusion:RGNT is a new classified central nervous system tumor corresponding to WHO grade I,codified by 2007 classification published by WHO.The immunohistochemical findings may be helpful for the differential diagnosis.