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目的探讨遗传性肾癌的诊断和治疗方法。方法回顾性分析11例遗传性肾癌患者的临床资料,其中男8例、女3例,年龄32～67岁,平均48岁；4例为双侧肾癌,4例为多发肾癌。2例诊断为希佩尔-林道病综合征,6例诊断为家族性肾透明细胞癌,3例诊断为遗传性乳头状肾癌。10例患者行保留肾单位的手术和(或)肾癌根治术,1例未手术。结果随访12～114个月,4例发生肿瘤复发,1例死于肿瘤转移,2例死于其他原因,4例无瘤生存。结论遗传性肾癌发病年龄较早,肿瘤双侧、多中心发病率较高,应尽量行保留肾单位手术。
Objective To investigate the diagnosis and treatment of hereditary renal cancer. Methods The clinical data of 11 patients with hereditary renal cell carcinoma were retrospectively analyzed. There were 8 males and 3 females, aged 32-67 years, with an average of 48 years old. Four patients had bilateral renal cell carcinoma and 4 patients had multiple renal cell carcinoma. 2 cases were diagnosed as Hippel-Lindau’s disease, 6 cases were diagnosed as familial renal clear cell carcinoma and 3 cases were diagnosed as hereditary papillary renal cell carcinoma. Ten patients underwent nephron surgery and / or radical nephrectomy, and one patient was not surgically treated. Results The patients were followed up for 12 to 114 months. Tumor recurrence occurred in 4 patients, 1 patient died of tumor metastasis, 2 died of other causes and 4 patients survived without any tumor. Conclusion The age of onset of hereditary renal cell carcinoma is earlier, and the incidence of bilateral and multicentric tumors is higher. Renal unit surgery should be reserved as far as possible.