Central nervous system neuroblastic tumor with FOXR2 activation presenting both neuronal and glial differentiation: a case report
Central nervous system neuroblastic tumor with FOXR2 activation presenting both neuronal and glial differentiation: a case report
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DOI：
10.1007/s10014-020-00370-2
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发表时间：
2020-06-13
影响因子：
3.3
通讯作者：
Sugita, Yasuo
中科院分区：
文献类型：
--
作者：
Furuta, Takuya;Moritsubo, Mayuko;Sugita, Yasuo
中枢神经系统神经母细胞瘤（CNS NB）的一个子集，罕见的原发性胚胎性中枢神经系统肿瘤，已包含在具有FOXR 2激活的中枢神经系统NB（CNS NB-FOXR 2）中，通常显示原始神经元结构和偶尔的神经细胞分化。在此，我们报告一例罕见的3岁女性中枢神经系统胚胎性肿瘤的罕见形态与FOXR 2激活呈现双向分化的神经细胞小原始细胞和星形胶质细胞梭形细胞，这两者都是阳性的突触素和GFAP。超微结构显示肿瘤细胞内存在突触前结构和中间纤维，提示胶质神经元分化。本例提示中枢神经系统神经母细胞瘤可能分化为神经元和神经胶质细胞。
A subset of central nervous system neuroblastomas (CNS NB), rare primary embryonal CNS tumors, has been encompassed in CNS NB withFOXR2activation (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 withFOXR2activation 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.