Comparative analysis of renal tumor patients with van Hippel-Lindau disease and those with sporadic renal cell cancers / 中华泌尿外科杂志

ABSTRACT

Objective To investigate the clinical features of renal tumor patients with yon Hip-pel-Lindau (VHL) disease. Methods Clinical data of 9 renal tumor cases with VHL disease were analyzed and reviewed. The renal tumor characteristics were compared with data of 46 sporadic renal cell cancer (SRCC) cases during the same period. Results In the 9 renal tumor cases with VHL dis-ease presented, significant statistical differences were found in the aspects of bilateralness (7 vs 0), ipsilateral multicentricity(5 vs 2)and cystic growth pattern(6 vs 1) compared with SRCC (P＜0.01). During the average follow-up period of 54 months, no significant difference was found in the median survival between VHL disease patients and SRCC patients(136 vs 42 months). However, a better tendency of VHL disease over SRCC was found in survival curve study. Conclusions VHL disease should be considered when renal tumors present bilateral, multicentric or with cystic growth pattern. Management should be individualized with close watchful waiting in combination with nephron sparing surgery. Nephrectomy should be avoided when possible.