副神经节瘤

以下是引用shandongzhang在2007-7-21 10:23:00的发言：  副神经节瘤

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This is a very circumscribed, partially cystic neoplasm in the posterior frontal brain parenchyma in a 18-year-old woman who presented with seizure disorder. My first choice of pathologic diagnosis, based on the photos presented, is WHO grade I gangliocytoma. Primary well differentiated neuronal tumors in the brain can display various architectural patterns in histopathology. Central neurocytoma in the ventricular wall is one unique example supratentorially, while cerebellar liponeurocytoma and dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) are two other intraftentorially. Gangliocytomas are a group of neuronal tumors consisting exclusively of large dysplastic neurons or ganglion cells. Less known are gangliocytomas with a paraganglioma-like nesting architectural pattern as seen here. Though reminiscent of many neuroendocrine neoplasms (including paraganglioma) outside of the central nervous system, the nesting pattern seen in this case is not associated with S100-positive sustentacular cells. GFAP-positive cells are sometimes seen in gangliocytomas, but never in paragangliomas. Primary paragangliomas in brain parenchyma (away from the jugular foramen at skull base) are extremely rare, and most are metastatic in nature. Therefore, I would not put paraganglioma (metastatic or primary) on top of the list of differential disgnosis for this case. 

 试译mjma老师的发言：有不当处请各位老师指正。

该肿瘤发生于18岁女性左额叶后脑实质，边界清楚、部分囊性，临床有抽搐发作。根据上传图片，我首先考虑神经节细胞瘤（WHO I级）。脑原发分化好的神经元肿瘤组织学可以呈现多种结构形态。脑室的中枢神经细胞瘤是发生于幕上的一种神经元肿瘤特殊形式，而小脑脂肪神经细胞瘤和小脑发育不良神经节细胞瘤（Lhermitte-Duclos病）是另外两种发生于幕内的神经元肿瘤。神经节细胞瘤是一种含独特的发育不良神经元或神经节细胞的神经元肿瘤。象图中所示有副神经节瘤样巢状结构的神经节细胞瘤少见。然而，许多中枢神经系统以外的神经内分泌肿瘤(包括副神经节瘤)，巢状结构中的支持细胞S－100并不阳性。神经节细胞瘤中有些细胞（非肿瘤性星形细胞）GFAP可以阳性，但副神经节瘤GFAP往往阴性。脑实质原发副神经节瘤（除了颅底颈静脉孔）相当少见，许多都是转移来的。因此，本例我首先没有考虑副神经节瘤（不管是原发还是转移）。

 听君细分析，胜读１０年书，真是好病例

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 请留意箭头标识，不知有何新意见？

请问马老师：whether primary neuroglial tumors were strongly positive for Syn、NSE(neuronal markers) and s-100?

 补充：IHC-- Ki67仅个别细胞阳性。

以下是引用yourself在2007-7-23 21:04:00的发言：  请留意箭头标识，不知有何新意见？ 请问马老师：whether primary neuroglial tumors were strongly positive for Syn、NSE(neuronal markers) and s-100?

这些"嗜酸性小体"除见于毛细胞星形细胞瘤\多形性黄色星形细胞瘤外还可见于Gangliocytoma/Ganglioglioma,结合免疫组化能否诊断Ganglioglioma?

以下是引用shandongzhang在2007-7-23 22:53:00的发言： 以下是引用yourself在2007-7-23 21:04:00的发言: 请留意箭头标识，不知有何新意见？请问马老师：whether primary neuroglial tumors were strongly positive for Syn、NSE(neuronal markers) and s-100? 这些"嗜酸性小体"除见于毛细胞星形细胞瘤\多形性黄色星形细胞瘤外还可见于Gangliocytoma/Ganglioglioma,结合免疫组化能否诊断Ganglioglioma?

Thanks to "yourself" for labeling the structures with arrows for me to study. Though I am not 100% certain, these highlighted eosinophilic structures may indeed be eosinophilic granular bodies. To be certain, I would like to see them at high power (400x). If they are, their presence is strongly supportive of gangliocytoma and against paraganglioma. Primary neuroglial tumors are usually S100-positive to varying extent. NSE immunoreactivity is also often seen in variable extent in various neuroglial neoplasms. However, only those with neuronal differentiation (and well differentiated choroid plexus papilloma and normal choroid plexus) will show immunoreactivity to antibodies against synaptophysin. 

I had some difficulty interpreting the GFAP photo. There are many GFAP-positive cells in the photo; in other word, they do not have glial morphology on routine HE stain. And, they are morphologically indistinguishable from the many cells that are strongly synaptophysin- and NSE-positive. In such case, I would not classify this as a ganglioglioma simply because some neoplastic cells are GFAP-positive. I would still prefer to classify this as a gangliocytoma (perhaps with aberrant expression of GFAP in some cells).

 谢谢马老师为精彩讲解！！

我们的考虑诊断有三个：1.毛细胞性星形细胞瘤（pilocytic astrocytoma）WHO I级

2.神经节细胞瘤（gangliocytoma ）WHO I级

3.神经节胶质瘤（ganglioglioma）WHO I级

最后我们认为诊断：毛细胞性星形细胞瘤（pilocytic astrocytoma）WHO I级较为妥当。

原因我们认为神经节细胞瘤或神经节胶质瘤这两种肿瘤中常常可见“细胞体积较大的神经节细胞”，而单一性“小细胞型神经节细胞”构成的肿瘤较少见，我们诊断上没有经验，另该肿瘤GFAP强阳性似乎更支持成胶质性肿瘤，而非成神经元性肿瘤。

再次谢谢马老师的精彩点评！！

 本例我认为诊断 gangliocytoma比较合适.

原因很简单, 毛细胞性星形细胞瘤不可能如此强阳性表达SYN和NSE.

有条件的话可以做做NF和Neu-N.

 马老师的讲解太精彩了，谢谢