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Central nervous system neuroblastic tumor with FOXR2 activation presenting both neuronal and glial differentiation: a case report.
Brain Tumor Pathology ( IF 3.3 ) Pub Date : 2020-06-13 , DOI: 10.1007/s10014-020-00370-2
Takuya Furuta 1 , Mayuko Moritsubo 1 , Hiroko Muta 1 , Motohisa Koga 2 , Satoru Komaki 2 , Hideo Nakamura 2 , Motohiro Morioka 2 , Koichi Ohshima 1 , Yasuo Sugita 3
Affiliation  

A subset of central nervous system neuroblastomas (CNS NB), rare primary embryonal CNS tumors, has been encompassed in CNS NB with FOXR2 activation (CNS NB-FOXR2) and usually shows the primitive neuronal architecture and occasional neurocytic differentiation. Here, we report a rare case of 3-year-old female with uncommon morphology of CNS embryonal tumor with FOXR2 activation presenting bidirectional differentiation to neurocytic small primitive cells and astrocytic spindle cells both of which are positive for synaptophysin and GFAP. Ultrastructural study also showed that there were presynaptic structure and intermediate filament in the tumor cells, suggesting glioneuronal differentiation. This case indicates the possibility of CNS neuroblastic tumor to differentiate neuronal and glial lineages.

中文翻译:

具有FOXR2激活的中枢神经系统神经母细胞瘤表现神经元和神经胶质分化:一例。

中枢神经系统神经母细胞瘤(CNS NB)的子集,罕见的原发性胚胎CNS肿瘤,已包含在具有FOXR2激活作用的CNS NB中(CNS NB- FOXR2),通常显示原始的神经元结构和偶尔的神经细胞分化。在这里,我们报告了一个罕见的3岁女性,FOXR2的中枢神经系统胚胎肿瘤形态不常见的情况激活呈现双向分化的神经细胞小原始细胞和星形细胞梭形细胞,两者都是突触素和GFAP阳性。超微结构研究还显示,肿瘤细胞中存在突触前结构和中间丝,提示神经胶质神经元分化。这种情况表明中枢神经系统成神经细胞肿瘤分化神经元和神经胶质谱系的可能性。
更新日期:2020-06-13
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