Walther M M, Lubensky I A, Venzon D, Zbar B, Linehan W M
Urologic Oncology Section, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.
J Urol. 1995 Dec;154(6):2010-4; discussion 2014-5.
We sought to describe the earliest renal lesions in patients with von Hippel-Lindau disease to gain insight into the genesis of renal neoplasms in this condition.
Grossly normal renal parenchyma from von Hippel-Lindau disease patients was examined microscopically and compared to findings in similar tissues obtained from patients with sporadic renal cancer and from autopsy subjects with no renal disease.
Microscopic renal cystic and solid neoplasms containing only clear cell cytological features were found in patients with von Hippel-Lindau disease. Benign cysts with clear cell cytological features were found only in patients with von Hippel-Lindau disease. Benign cysts lined by cuboidal cells with eosinophilic cytoplasm, similar to renal tubular cells, were present only in patients with renal cancer. The extrapolated number of clear cell lesions in an average kidney with von Hippel-Lindau disease was estimated as 1,100 cysts (benign or atypical) with clear cell lining and 600 clear cell neoplasms.
These findings support the hypothesis that abnormalities in the von Hippel-Lindau gene in a kidney results in the cytological cell type of clear cell renal cancer as the initial product of cellular transformation.
我们试图描述冯·希佩尔-林道病患者最早出现的肾脏病变，以深入了解这种情况下肾肿瘤的发生机制。
对冯·希佩尔-林道病患者大体正常的肾实质进行显微镜检查，并与散发性肾癌患者及无肾脏疾病的尸检对象的类似组织的检查结果进行比较。
在冯·希佩尔-林道病患者中发现了仅具有透明细胞细胞学特征的显微镜下可见的肾囊性和实性肿瘤。仅在冯·希佩尔-林道病患者中发现了具有透明细胞细胞学特征的良性囊肿。仅在肾癌患者中存在由具有嗜酸性细胞质的立方体细胞衬里的良性囊肿，类似于肾小管细胞。据估计，平均患有冯·希佩尔-林道病的肾脏中透明细胞病变的数量为1100个具有透明细胞衬里的囊肿（良性或非典型性）和600个透明细胞瘤。
这些发现支持以下假设：肾脏中冯·希佩尔-林道基因的异常导致透明细胞肾癌的细胞学细胞类型作为细胞转化的初始产物。
