首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Neuronal differentiation associated with Gli3 expression predicts favorable outcome for patients with medulloblastoma
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Neuronal differentiation associated with Gli3 expression predicts favorable outcome for patients with medulloblastoma

机译:与Gli3表达相关的神经元分化预测髓母细胞瘤患者的预后良好

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摘要

Medulloblastoma (MB) is a malignant cerebellar tumor arising in children, and its ontogenesis is regulated by Sonic Hedgehog (Shh) signaling. No data are available regarding the correlation between expression of Gli3, a protein lying downstream of Shh, and neuronal differentiation of MB cells, or the prognostic significance of these features. We re-evaluated the histopathological features of surgical specimens of MB taken from 32 patients, and defined 15 of them as MB with neuronal differentiation (ND), three as MB with both glial and neuronal differentiation (GD), and 14 as differentiation-free (DF) MB. Gli3-immunoreactivity (IR) was evident as a clear circular stain outlining the nuclei of the tumor cells. The difference in the frequency of IR between the ND+GD (94.4%) and DF (0%) groups was significant (P<0.001). The tumor cells with ND showed IR for both Gli3 and neuronal nuclei. Ultrastructurally, Gli3-IR was observed at the nuclear membrane. The overall survival and event-free survival rates of the patients in the ND group were significantly higher than those in the other groups. The expression profile of Gli3 is of considerable significance, and the association of ND with this feature may be prognostically favorable in patients with MB.
机译:髓母细胞瘤(MB)是儿童所患的恶性小脑肿瘤,其声波受Sonic Hedgehog(Shh)信号调节。关于Gli3(一种位于Shh下游的蛋白)的表达与MB细胞的神经元分化之间的相关性,或这些特征的预后意义,尚无相关数据。我们重新评估了32例MB手术标本的组织病理学特征,并将其中15例定义为神经元分化(ND)的MB,3例同时具有神经胶质和神经元分化(GD)的MB和14例无分化的MB (DF)MB。 Gli3免疫反应性(IR)明显表现为清晰的圆形染色,勾勒出肿瘤细胞的细胞核。 ND + GD(94.4%)和DF(0%)组的IR频率差异显着(P <0.001)。 ND肿瘤细胞对Gli3和神经元核均显示IR。超微结构,在核膜上观察到Gli3-IR。 ND组患者的总生存率和无事件生存率显着高于其他组。 Gli3的表达谱具有相当重要的意义,ND与该特征的相关性在MB患者的预后上可能是有利的。

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