VHL病伴发双侧肾癌3例报告并文献复习

Von Hippel-Lindau disease accompanying bilateral renal carcinoma:A report of 3 cases and literature review

目的初步探讨以冯.希佩尔.林道(Von Hippel-Lindau,VHL)病并发双侧肾癌的发病特点、诊断及治疗。方法回顾分析2007年2月-2011年6月解放军第309医院收治的3例VHL病并发双侧肾癌的患者,并结合文献复习。结果 3例中1例有家族史,先后行右侧保留肾单位手术,左侧腹腔镜下肾癌根治性切除,病理提示为透明细胞癌,术后规律血液透析,等待肾移植;另2例为多发病变,双侧肾癌伴双侧多发肾囊肿,2例均选择双侧保留肾单位手术和肾囊肿去盖术,术后病理提示为透明细胞癌,肾功能正常。结论 VHL病肾癌有独特的临床特征,不同于散发性肾癌,诊断主要依靠MRI和CT;治疗主要依靠手术,术后易复发,因累及多脏器,需要多学科给予综合治疗。

Objective To study the onset characteristics,diagnosis and treatment of von Hippel-Lindau（VHL） disease accompanying bilateral renal carcinoma.Methods Clinical data about 3 patients with VHL disease accompanying bilateral renal carcinoma admitted to our hospital from February 2007 to June 2011 were retrospectively analyzed with the related literature reviewed.Results One of the 3 patients had a family history and underwent laparoscopic radical resection of carcinoma in the left kidney with the renal unit of the right kidney preserved.Pathological examination suggested that it should be a clear cell carcinoma and hemodialysis should be performed for renal transplantation.The other two patients had multiple pathological lesions,including bilateral renal carcinoma accompanying multiple renal cysts,and underwent selective bilateral nephron-sparing and renal cyst surgery.Pathological examination showed that it was a clear cell carcinoma with normal renal function.Conclusion VHL disease with unique clinical features is different from sporadic renal cancer,its diagnosis mainly relies on MRI and CT,and operation is the main procedure for it.It is easy to relapse with many organs involved,thus needing comprehensive and multidisciplinary treatment.