Von Hippel—Lindau病肾癌的临床特征分析

目的 探讨Von Hippel-Lindau（VHL）病肾癌的临床特点。方法回顾分析28例VHL病肾癌患者的临床资料。就初诊年龄、肿瘤部位、同时或异时癌、肿瘤的组织病理等与散发性肾癌进行比较。结果VHL肾癌初诊年龄44．6岁，双肾癌15例、多灶性肾癌16例、伴双侧多发肾囊肿20例。共切除87个实性肿瘤。术后病理：透明细胞癌86个，Fuhrman分级Ⅰ级73个、Ⅱ级12个、Ⅲ级1个；钙化结节1个。TNM分期ⅠA期、ⅠB期、Ⅱ期、Ⅲ期分别为8例、7例、8例和1例。与散发性肾癌组相比，VHL病肾癌组患者发病年龄早（P〈0．05），双肾多灶性肾癌及伴双侧多发肾囊肿比例高（P〈O．001），高级别肿瘤比例低（P〈O．05）。结论VHL病肾癌不同于散发性肾癌，有其独特的临床病理特征，这对该病诊断治疗具有一定指导价值。

Clinicopathological characteristics of renal cell carcinoma associated with von Hippel-Lindau disease

Objective To explore the Hippel-Lindau disease （VHLD）. Methods clinicopathological features of the renal cell carcinoma （RCC） associated with von Clinical data of 28 cases of RCC associated with VHLD were reviewed and analyzed. Results Among the 28 cases, the mean age of first RCC at diagnosis was 44.6 years and there were 15（53.6%） with bilateral RCCs, 16（57.1%） with multifocal RCCs, 20（71.4%） with bilateral multiple renal cysts, 22（78.6%） with central nervous system hemangioblastomas or retinal angiomas, and 18（64.3%） with multiple pancreatic cysts. 87 solid lesions and 72. cystic lesions were removed. Pathological results confirmed 86 renal clear cell carcinomas. The Fuhrmans grade was grade I in 73 tumors, grade Ⅱ in 12 and grade Ⅲ in 1. The TNM stage was Tla in 8 cases, Tlb in 7 cases, T2 in 8 cases and T3 in 1 case. Compared with sporadic, the RCC in VHLD showed an earlier occurrence （P〈0. 05）, a more frequent association with renal cysts, multifocal and bilateral tumors （P〈0. 001）, and a more frequent low grade histology（P〈0.05）. Conclusion The renal lesions in von Hippel-Lindau disease have specific clinicopathological features which are of value in the diagnosis and treatment.