NHERF1/EBP50 and NF2 as diagnostic markers for choroid plexus tumors.
NHERF1/EBP50 and NF2 as diagnostic markers for choroid plexus tumors.
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DOI：
10.1186/s40478-016-0329-0
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发表时间：
2016-05-27
影响因子：
7.1
通讯作者：
Raisanen JM
中科院分区：
文献类型：
--
作者：
Georgescu MM;Mobley BC;Orr BA;Shang P;Lehman NL;Zhu X;O'Neill TJ;Rajaram V;Hatanpaa KJ;Timmons CF;Raisanen JM
连接蛋白NHERF1(钠/氢交换因子-3调节因子-1)及其相关的Ezrin-Radisin-moesin-merlin/urofimin-2(ERM-NF2)家族蛋白是上皮细胞形态发生所必需的，并与肿瘤的进展有关。NHERF1在室管膜细胞中表达，是室管膜瘤的一个高度敏感的诊断标记物，它标记膜的极性结构。由于NHERF1和ERM-NF2蛋白在脉络丛(CP)细胞中呈现极化表达，我们测试了它们在CP肿瘤中的诊断价值。在43例成人和儿童乳头状肿瘤中，NHERF1免疫组织化学染色显示CP乳头状瘤(WHO分级I级)顶端质膜呈强阳性染色，CP癌(WHO分级III级)呈胞浆表达。Ezrin和Moesin的染色相似，但不太明显。NHERF1还标记了松果体区的乳头状肿瘤，呈微腔和局部顶膜模式，提示CP乳头状瘤和室管膜瘤之间的过渡形态。NF2可将所有级别的CP肿瘤与乳头状结构的转移性癌区分开来，在CP肿瘤中表现为偏振的膜染色。NHERF1和NF2免疫组织化学显示，与常用的细胞角蛋白和Kir7.1等标记物相比，NHERF1和NF2对CP肿瘤的敏感性和特异性更高，是鉴别诊断中枢神经系统乳头状肿瘤的可靠工具。本文的在线版本(doi：10.1186/s40478-0160329-0)包含补充材料，授权用户可以使用。
The adaptor protein NHERF1 (Na/H exchanger-3 regulatory factor-1) and its associated ezrin-radixin-moesin-merlin/neurofibromin-2 (ERM-NF2) family proteins are required for epithelial morphogenesis and have been implicated in cancer progression. NHERF1 is expressed in ependymal cells and constitutes a highly sensitive diagnostic marker for ependymoma, where it labels membrane polarity structures. Since NHERF1 and ERM-NF2 proteins show polarized expression in choroid plexus (CP) cells, we tested their diagnostic utility in CP neoplasms. NHERF1 immunohistochemistry in 43 adult and pediatric tumors with papillary morphology revealed strong apical plasma membrane staining in CP papilloma (WHO grade I) and cytoplasmic expression in CP carcinoma (WHO grade III). Ezrin and moesin showed similar but less distinctive staining. NHERF1 also labeled papillary tumors of the pineal region in a microlumen and focal apical membrane pattern, suggestive of a transitional morphology between CP papilloma and ependymoma. CP tumors of all grades could be differentiated from metastatic carcinomas with papillary architecture by NF2, which showed polarized membranous staining in CP tumors. NHERF1 and NF2 immunohistochemistry showed enhanced sensitivity and specificity for CP tumors compared to commonly used markers, including cytokeratins and Kir7.1, emerging as reliable diagnostic tools for the differential diagnosis of papillary tumors of the central nervous system. The online version of this article (doi:10.1186/s40478-016-0329-0) contains supplementary material, which is available to authorized users.