摘要
目的 探讨vonHippel Lindau(VHL)病肾脏损害的病理特点及VHL多发性肾癌的治疗。 方法 回顾性分析 6例同一VHL病家族伴有肾脏损害患者的临床及病理资料 ;1例为双肾多发性囊肿 ,5例为双侧肾癌伴多发性囊肿。 结果 5例双肾癌 10侧肾脏中 ,行保留肾单位手术 5侧 ,肾癌根治术 3侧 ,带瘤随访 2侧。 1例多发肾囊肿者仅随诊观察。术后病理实质性肿块均为透明细胞癌。Fuhrman分级结果 :Ⅰ级 14枚 ,Ⅱ级 6枚。TNM病理分期 :T1N0 M0 4例、T2 N0 M0 1例。切除 14枚可疑的肾囊肿 ,术后病理恶性囊肿 1枚 ,余为单纯囊肿。术后随访 9～ 113个月 ,平均 4 7个月 ,5例患者均存活且无肿瘤复发及远处转移 ;5例肾功能正常 ,1例肾功能较术前差 ,但无需血透治疗。 结论 VHL病中肾脏损害包括肾癌及肾囊肿 ,常为双侧多发性 ,肿瘤为透明细胞癌且分级分期低。
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.
出处
《中华泌尿外科杂志》
CAS
CSCD
北大核心
2004年第11期756-759,共4页
Chinese Journal of Urology
