VHL基因是 1993年克隆成功的重要抑癌基因 ,其编码的蛋白产物pVHL可在转录因子调控、蛋白水解等多层次上影响肿瘤的生物学活性。VHL基因突变与遗传性及散发性肾细胞癌的发生、病理分型、分期及检测治疗等各方面均有密切关系。VEGF的高表达则是VHL相关疾病的一个显著特征 ,抗血管形成药物有望成为治疗VHL病的新手段 VHL gene is an important tumor suppressor gene successfully cloned in 1993. Its protein product pVHL can affect tumor biological activity at transcriptional level and proteolysis. VHL gene mutations and hereditary and sporadic renal cell carcinoma, pathological classification, staging and detection of treatment are closely related. High expression of VEGF is a prominent feature of VHL-related diseases, anti-angiogenic drugs is expected to become a new approach to the treatment of VHL disease