Altered expression of key cell cycle regulators in renal cell carcinoma associated with Xp11.2 translocation
Altered expression of key cell cycle regulators in renal cell carcinoma associated with Xp11.2 translocation
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DOI：
10.1016/j.prp.2009.01.005
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发表时间：
2009-01-01
影响因子：
2.8
通讯作者：
Mueller-Hoecker, J.
中科院分区：
文献类型：
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作者：
Barroca, H.;Castedo, S.;Mueller-Hoecker, J.
肾细胞癌（RCC）是儿科人群中一种罕见的肿瘤。最近，已经描述了一种表型和遗传上不同的肾癌，主要流行于儿童并与 Xp11.2 易位或 TFE3 基因融合相关。研究表明，在这种 RCC 亚型中，存在细胞周期蛋白 D1、细胞周期蛋白 D3 和 p21((wafl/cip1)) 的积累。本研究的目的是弄清楚在我们科室最近诊断出的两例儿科肾细胞癌（一例透明细胞型肾细胞癌和一例 TFE3 阳性肾细胞癌）中，这些特征是否确实是后一种肿瘤所特有的，或者是否发生在儿科肾细胞癌中，无论肿瘤类型如何。两种情况均进行以下免疫染色：Ki67、p16(ink4a)、p21((wafl/cip1))、p27(kip1)、p53、p63、mdm2、细胞周期蛋白 D1、细胞周期蛋白 D3、TFE3、CD10、波形蛋白、E-钙粘蛋白和 RCC 抗原。我们在 TFE3 阳性癌症中观察到 p21((wafl/cip1))、细胞周期蛋白 D1 和细胞周期蛋白 D3 的强烈免疫反应，而没有 p53、p16、p27 kipl 和 mdm2 的表达，而在经典 RCC 中观察到的免疫表达谱与透明细胞、成人型 RCC 相似。我们的研究证实，TFE3 阳性癌症 RCC 表现出细胞周期失调，显然与患者的年轻年龄无关。 (C) 2009 爱思唯尔有限公司。版权所有。
Renal cell carcinoma (RCC) is a rare tumor in the pediatric population. Recently, a phenotypically and genetically distinct kidney carcinoma, mainly prevalent in children and associated with an Xp11.2 translocation or TFE3 gene fusion, has been described. It has been advanced that in this subtype of RCC, there is an accumulation of cyclin D1, cyclin D3, and p21((wafl/cip1)). The aim of the present study was to figure out in two pediatric RCC recently diagnosed in our department (one clear cell-type RCC and one TFE3-positive RCC) whether those features are indeed specific of the latter tumor or occur in pediatric RCC irrespective of the tumor type. The following immunostains were performed in both cases: Ki67, p16(ink4a), p21((wafl/cip1)), p27(kip1), p53, p63, mdm2, cyclin D1, cyclin D3, TFE3, CD10, vimentin, E-cadherin, and RCC-antigen. We observed in the TFE3-positive carcinoma an intense immunoreaction for p21((wafl/cip1)), cyclin D1, and cyclin D3, without expression for p53, p16, p27 kipl, and mdm2, whereas the immunoexpression profile observed in the classic RCC was similar to that of clear cell, adult-type RCC.Our study confirms that TFE3-positive RCC exhibits a deregulation of the cell cycle apparently unrelated to the young age of the patients. (C) 2009 Elsevier GmbH. All rights reserved.