Von-Hippel-Lindau病并发肾癌2例

发布时间：2018-07-13 18:08

【摘要】：目的探讨Von-Hippel-Lindau(VHL)病并发肾癌等疾病的临床特点。方法回顾分析山东大学第二医院收治的2例VHL病并发肾癌的患者,并结合文献复习。结果 2例中1例有家族史,行右肾癌根治术,病理显示为肾透明细胞癌,术后随访1年,未见复发;1例无相关疾病家族史,先行右肾癌根治术,病理显示右肾多房性囊性肾细胞癌,右肾上腺嗜铬细胞瘤,术后4年左肾及左侧肾上腺出现肿瘤复发,左肾肿瘤穿刺病理提示肾透明细胞癌,给予分子靶向药物索拉菲尼治疗,现规律透析。结论 VHL病是涉及多个系统的肿瘤综合征,临床较为少见,其并发肾癌的特征不同于散发性肾癌;早诊断、早进行个体化治疗可改善患者晚期生存质量。
[Abstract]:Objective to investigate the clinical features of Von-Hippel-Lindau disease (VHL) complicated with renal cell carcinoma (RCC). Methods two patients with VHL complicated with renal cell carcinoma were reviewed retrospectively. Results one case with family history underwent radical resection of right renal cell carcinoma (RRCC), and the pathological findings were clear cell carcinoma of kidney (RCC). After one year of follow-up, no recurrence was found in 1 case without any family history of related diseases. Radical resection of RRCC was performed first. Pathological findings showed that right renal multilocular cystic renal cell carcinoma, right adrenal pheochromocytoma, left kidney and left adrenal gland recurred 4 years after operation, left renal tumor puncture pathology suggested renal clear cell carcinoma, and was treated with molecular targeted drug Solafenil. Now regular dialysis. Conclusion VHL disease is a tumor syndrome involving multiple systems, and its clinical features are different from those of sporadic renal cell carcinoma. Early diagnosis and early individualized treatment can improve the quality of life of patients with advanced stage.
【作者单位】：山东大学第二医院泌尿外科;
【基金】：山东省科技发展计划(2014GSF118150)
【分类号】：R737.11

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