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冯·希佩尔-林道病中转移性肾细胞癌至成血管细胞瘤

Metastatic renal cell carcinoma to hemangioblastoma in von Hippel-Lindau disease.

作者信息

Polydorides Alexandros D, Rosenblum Marc K, Edgar Mark A

机构信息

Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University, New York, NY, USA.

出版信息

Arch Pathol Lab Med. 2007 Apr;131(4):641-5. doi: 10.5858/2007-131-641-MRCCTH.

DOI:10.5858/2007-131-641-MRCCTH
PMID:17425399
Abstract

A case of metastatic renal cell carcinoma (RCC) to a capillary hemangioblastoma (HAB) of the central nervous system in a 52-year-old woman with von Hippel-Lindau (vHL) syndrome is described. We review the literature on metastatic RCC to HAB, summarize the histologic and immunohistochemical features that can distinguish between the 2 tumors, and comment on the significance of such a finding in terms of the clinical diagnosis of vHL. We found the expression of CAM 5.2, RCC antigen, and CD10 to be strong in RCC and absent in HAB and, conversely, staining with Leu-7, neural cell adhesion molecule, and inhibin-alpha was present in HAB but weak or absent in RCC. These antibodies can be used to differentiate these entities, provided one is astute in recognizing the possibility of their coexistence.

摘要

本文描述了一例52岁患有冯·希佩尔-林道(vHL)综合征的女性,其转移性肾细胞癌(RCC)转移至中枢神经系统的毛细血管型血管母细胞瘤(HAB)。我们回顾了关于RCC转移至HAB的文献,总结了可区分这两种肿瘤的组织学和免疫组化特征,并就这一发现对vHL临床诊断的意义进行了评论。我们发现,CAM 5.2、RCC抗原和CD10在RCC中呈强表达而在HAB中缺失,相反,Leu-7、神经细胞黏附分子和抑制素-α在HAB中呈阳性染色而在RCC中弱表达或缺失。只要能敏锐地认识到它们共存的可能性,这些抗体可用于区分这些实体。

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