Renal pathological features in von Hippel-Lindau disease
- VernacularTitle:von Hippel-Lindau病的肾脏病理学特点
- Author:
Jin ZHANG
;
Yiran HUANG
;
Lianfang DU
- Publication Type:Journal Article
- Keywords:
Von Hippel-Lindau disease;
Renal cell carcinoma;
Renal cyst;
Pathology;
Nephron-sparing surgery
- From:
Chinese Journal of Urology
2001;0(11):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the features of renal lesions in von Hippel-Lindau (VHL) disease and the surgical intervention. Methods The clinical and pathological data from 6 patients with renal lesions in a large Chinese VHL disease kindred were reviewed individually.One patient had multifocal renal cysts,and 5 patients had bilateral renal cell carcinomas and multifocal renal cysts. Results Among 10 kidneys in the 5 cases of bilateral renal cell carcinomas, nephron-sparing surgery (NSS) was performed in 5,radical nephrectomy (RN) in 3 and observation in 2.After operation,the pathological findings indicated that all solid lesions were renal clear cell carcinoma, with one malignant cyst being detected.The Fuhrman's nuclear grade of the RCC was grade Ⅰ in 14 tumors,grade Ⅱ in 6 tumors.The TNM stage was T 1N 0M 0 in 4 cases and T 2N 0M 0 in 1 case.During a mean follow-up of 47 months (range,9 to 113 months),all patients were alive without evidence of RCC recurrence and metastasis.Five patients had normal renal function and 1 patient experienced an increase in serum creatinine but without dialysis. Conclusions The renal lesions in VHL disease include both cysts and carcinomas,which are often multifocal and bilateral.Almost all the tumors are of clear cell type with a low stage and grade.Nephron-sparing surgery may be justified and effective in the management of VHL patients.
