von Hippel—Lindau病肾肿瘤与散发性肾细胞癌临床比较分析  

Comparative analysis of renal tumor patients with von Hippel-Lindau disease and those with sporadic renal cell cancers

作　　者：张弋[1] 李进[1] 王健[1] 高伟[1] 崔昕[1] 欧彤文[1] 

机构地区：[1]首都医科大学宣武医院泌尿外科,北京100053

出　　处：《中华泌尿外科杂志》2008年第9期617-620,共4页Chinese Journal of Urology

摘　　要：目的探讨vonHippelLindau（VHL）病肾肿瘤的临床特征。方法回顾性分析9例VHL病肾肿瘤患者资料，其中男6例，女3例。平均年龄51岁。双侧肾肿瘤7例，单侧多中心表现5例，囊实性表现6例。同期散发性肾细胞癌患者46例，其中男30例，女16例。平均年龄55岁。肿瘤均为单发，单侧多中心表现2例，囊实性表现1例。分析2组患者在发病人群、肿瘤表现、疾病生存等方面的差异。结果2组患者性别、年龄比较差异无统计学意义（P〉0．05）。2组肾肿瘤双侧性、多中心性和囊实性表现比较差异均有统计学意义（P〈0．01）。VHL组平均随访54个月，死亡4例，死于远处转移3例、死于恶液质1例。VHL病肾肿瘤与散发性肾细胞癌患者中位生存时间分别为136、42个月，2组比较差异无统计学意义（P=0．251），但生存曲线显示VHL病肾肿瘤患者生存率呈现较高趋势。结论VHL病肾肿瘤临床表现与散发性肾细胞癌不同，临床上应根据临床特征正确诊断，避免肾切除术，采取保留肾单位手术结合严密影像学观察治疗策略。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 he 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. Nephreetomy should be avoided when possible.

关 键 词：von Hippel—Lindau病 肾肿瘤 癌 

分 类 号：R737.11[医药卫生—肿瘤] R749.3[医药卫生—临床医学]