Clear cell papillary renal cell carcinoma: a review.
Clear cell papillary renal cell carcinoma: a review.
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DOI：
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发表时间：
2014-10
影响因子：
1.4
通讯作者：
N. Kuroda;C. Ohe;F. Kawakami;S. Mikami;M. Furuya;K. Matsuura;M. Moriyama;Y. Nagashima;Ming Zhou-Ming
中科院分区：
文献类型：
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作者：
N. Kuroda;C. Ohe;F. Kawakami;S. Mikami;M. Furuya;K. Matsuura;M. Moriyama;Y. Nagashima;Ming Zhou-Ming
透明细胞（管状）乳头状肾细胞癌（CCP-RCC）作为肾细胞癌的独特亚型的疾病概念最近已被确立。这种肿瘤类型首先在终末期肾病的情况下被描述，但在散发的情况下更容易被识别和遇到。在本文中，我们概述了对该肿瘤的最新认识。肉眼可见，肿瘤边界清楚，肿瘤包膜发达。组织学上，肿瘤细胞呈立方形至低柱状细胞，细胞质透明，呈乳头状和管状乳头状结构。免疫组织化学显示，肿瘤细胞普遍表达细胞角蛋白7、CA9（杯状图案）、HIF-1、GLUT-1和高分子量细胞角蛋白，但AMACR、RCC Ma和TFE3阴性。大多数肿瘤中 CD10 呈阴性或局灶性阳性。从遗传学上来说，这种肿瘤不具有透明细胞肾细胞癌或乳头状肾细胞癌的特征。从预后来看，在之前报告的所有病例中，CCP-RCC 患者均表现出惰性行为。总之，虽然该肿瘤已被纳入最新的国际泌尿病理学会肾肿瘤分类，但疾病概念和临床行为均尚未完全阐明。大量患者的进一步发表将真正有助于了解这种肿瘤类型的生物学潜力和分子基础。
The disease concept of clear cell (tubulo) papillary renal cell carcinoma (CCP-RCC) as a distinct subtype of renal cell carcinoma has been recently established. First described in the setting of end stage renal disease, this tumor type is more frequently recognized and encountered in a sporadic setting. In this article, we provide an overview of the recent understanding of this tumor. Macroscopically, tumors are well circumscribed with well-developed tumor capsule. Histologically, the tumor cells are cuboidal to low columnar cell with clear cytoplasm and papillary and tubulo-papillary configuration. Immunohistochemically, tumor cells generally show diffuse expression for cytokeratin 7, CA9 (cup-shaped pattern), HIF-1, GLUT-1 and high molecular weight cytokeratin, but negative for AMACR, RCC Ma and TFE3. CD10 is negative or focally positive in most tumors. Genetically, this tumor has no characteristics of clear cell RCC or papillary RCC. Prognostically, patients with CCP-RCC behave in an indolent fashion in all previously reported cases. In conclusion, although this tumor has been integrated into recent International Society of Urologic Pathology Classification of renal neoplasia, both aspects of disease concept and clinical behavior are yet to be fully elucidated. Further publications of large cohorts of patients will truly help understand the biologic potential and the molecular underpinnings of this tumor type.