Chinese Journal of Urology, Vol. 30, No. 2, February 2009 Zhang Jin Huang Yiran Liu Dongming Zhou Lixin Xue Wei [Abstract] Objective To summarize the experience of diagnosis and treatment of renal cancer in yon Hippel-Lindau (VHL) disease. Methods There were 28 cases of VHL kidney cancer patients. There were 16 male cases and 12 female cases. The average age was 45 years. There were 15 cases of bilateral renal cancer (11 cases at the same time and 4 cases at different times) and 13 cases of unilateral renal cancer. VHL gene gun test was performed in 25 cases. 24 cases underwent kidney unit preservation surgery or radical kidney cancer surgery. There were 2 cases of watchful waiting and 2 cases of conservative treatment. The results were that all 25 subjects had VHL germline mutations. Among them, 14 patients were asymptomatic. 29 solid tumors were observed in 9 patients for a mean of 44 (12-86) months, with a mean tumor growth rate of 0.531 cm/year; at the end of observation, 19 (65.5%) tumors grew >3 cm, and only 1 tumor metastasized. 87 solid tumors were surgically removed in 24 cases, including 62 (71.3%) tumor enucleations, 1 tumor resection at the lower pole of the kidney, and 1 radical resection. In 24 cases, 87 solid tumors were surgically removed, including 62 tumor enucleations (71.3%), 1 lower pole resection and 24 radical nephrectomies. The postoperative pathology reported that all 24 cases were renal clear cell carcinoma. 8 cases of TNM stage T1a, 7 cases of T1b, 8 cases of T2, and 1 case of T3. There were 86 tumors, 73 Fuhrman grade I, 12 grade II, 1 grade III, and 1 calcified nodule. 28 patients were followed up for an average of 50 (5-237) months, 26 survived, 2 died, and 4 cases had local recurrence of tumor. Conclusion Genetic testing can detect asymptomatic VHI. patients early; VHI, disease. kidney cancer mostly grows slowly, and most tumors >3 cm do not metastasize and can be followed up and observed; preserving kidney unit surgery is a safe and effective method to treat kidney cancer of VHL disease. Zhang Jin, Department of Urology, Shanghai Renji Hospital [Keywords] von Hippel-Lindau disease; renal tumor; surgical procedure,.