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Von Hippel-Lindau(VHL)病是一种家族性肾肿瘤综合征,肾肿瘤表现为肾细胞癌,还可以累及中枢神经系统、视网膜、肾上腺、胰腺和附睾等。我院自1991年5月至2005年11月共诊断治疗 VHL 肾肿瘤患者9例,占同期诊断肾细胞癌的3.4%,现报告如下。临床资料1.一般资料:本组9例,其中男性6例,女性3例,年龄29～70岁,平均51岁。6例以血尿为首发症状,其中1例严重血尿;2例在接受神经系统手术前查体发现;1例因腰部胀痛就诊发现。双侧肾肿瘤5例,共14侧肾脏受累,多发11
Von Hippel-Lindau (VHL) disease is a familial renal tumor syndrome characterized by renal cell carcinoma, which can also affect the central nervous system, the retina, adrenal gland, pancreas and epididymis. Our hospital from May 1991 to November 2005 were diagnosed in patients with VHL renal tumors in 9 cases, accounting for the same period of diagnosis of renal cell carcinoma 3.4%, are as follows. Clinical data 1. General information: The group of 9 patients, 6 males and 3 females, aged 29 to 70 years, mean 51 years. Six cases had hematuria as the first symptom, of which one case had severe hematuria; two cases were found prior to the operation of the nervous system; one case was found due to lumbar pain. Bilateral renal tumors in 5 cases, a total of 14 side of the kidney involvement, multiple 11