Gong Da-xin, Wang Xia, Li Ze-liang, Jiang Yuan-jun, Sun Zhi-xi, Kong Chui-ze
Department of Urology, the First Affiliated Hospital of China Medical University, Shenyang 110001, China.
Zhonghua Wai Ke Za Zhi. 2006 Jul 15;44(14):963-5.
To evaluate the diagnosis and treatment of hereditary renal cell carcinoma.
Clinical data of 11 patients with hereditary renal cell carcinoma were analyzed retrospectively. Eight patients were male and 3 were female, age ranged from 32 to 67 (mean: age 48 years). Four cases were bilateral renal cell carcinoma, and 4 were multiple renal cell carcinoma. Two cases were diagnosed as Von Hippel-Lindau syndrome, 6 as familial clear cell renal cell cancer, and 3 as hereditary papillary renal carcinoma.
Ten patients performed nephron-sparing surgery and/or radical nephrectomy and 1 had no operation. The patients were followed up from 12 to 114 months. Tumor recurrence was observed in 4 patients, 1 patient died of tumor metastasis, and 2 died of other causes. Four patients survived free of tumor.
Hereditary renal carcinoma appears in the youth, and it is predominantly multiple and bilateral. Nephron-sparing surgery is the standard method of treatment for the patients.
评估遗传性肾细胞癌的诊断与治疗。
回顾性分析11例遗传性肾细胞癌患者的临床资料。8例为男性，3例为女性，年龄范围为32至67岁（平均年龄48岁）。4例为双侧肾细胞癌，4例为多发性肾细胞癌。2例被诊断为冯希佩尔-林道综合征，6例为家族性透明细胞肾细胞癌，3例为遗传性乳头状肾细胞癌。
10例患者接受了保留肾单位手术和/或根治性肾切除术，1例未进行手术。患者随访12至114个月。4例患者出现肿瘤复发，1例患者死于肿瘤转移，2例死于其他原因。4例患者无瘤生存。
遗传性肾癌发病于青年期，多为多发性和双侧性。保留肾单位手术是患者的标准治疗方法。
