von Hippel-Lindau病并发肾癌的诊断和治疗

The diagnosis and treatment of renal cell carcinoma in von Hippel-Lindau disease

目的 提高vonHippel Lindau病 (VHL)并发肾癌的诊断和治疗水平。 方法 回顾分析 7例VHL病并发肾癌患者临床资料并结合文献复习讨论。男 4例 ,女 3例 ,平均年龄 42岁。手术治疗 6例 ,共切除肿瘤 3 4个 ,其中 5个肾脏行肿瘤切除术 ,切除肿瘤 13个 ;切除肾脏 4个 ,发现肿瘤2 1个。 结果 随访 8～ 76个月 ,平均 45个月。 1例术后 2 6个月肾脏肿瘤复发 ,行肾脏切除。 1例双肾切除者 ,半年后行肾移植 ,随访 18个月未见肿瘤复发 ,移植肾功能良好。 4例患者肿瘤无复发和转移 ,肾功能正常。 1例患者拒绝治疗 ,随访 2 5个月肿瘤局部有进展 ,未发现远处转移。 结论 肾癌是VHL病主要病变之一 ,具有多中心、双侧发病、易复发等特点。薄层CT是主要的诊断和随访手段。手术治疗方式包括双肾切除 ,肾肿瘤剜除和肾部分切除等 ,保留肾单位术后应密切随访 。

Objective To study diagnosis and treatmen t of the renal cell carcinoma in von Hippel-Lindau disease. Methods Analyzed 7 cases of clinical date,and reviewed the literature.Of the 7 patients,4 were male,3 were female,with an average age of 42.Six patients und erwent renal surgery,and totally 34 tumors were resected(13 tumors from 5 conser vative renal surgery, the other 21 tumors from 4 radical nephrectomy). Results The mean follow-up for these patients was 45 months (ran ge,8～76 months).1 patient with a recurrent renal mass noted at 26 months after renal surgery underwent radical nephrectomy.1 patient,who had undergone bilatera l nephrectomy,received allo-renal transplantation at 6 months after surgery.Fol lowed up for 18 months,norecurrence was found,and allograft’s function was norm al.4 patients were tumor free and had adequate renal function.1 patient,who had refused any treatment,was followed-up for 25 months,the lesion was local advan ced without metastasis. Conclusions The characterization of renal cell carcinoma in von Hippel-Lindau disease may include multicentral, bilateral lesions and with the high risk of recurrence.The diagnosis and follow -up are mainly by CT scan.The surgery include radical nephrectomy,enucleation,p artial nephrectomy.A regular follow-up is important after conservative renal su rgery.