Von Hippel-Lindau综合征并发肾癌二例诊治分析  

Clinical diagnosis and treatment of renal cell carcinoma associated with von Hippel-Lindau syndrome: two cases report

作　　者：林向楠 高振利[1] 金海荣[1] 张鹏[1] 石磊[1] LIN Xiangnan;GAO Zhenli;JIN Hairong;et al(Department of Urology, the Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai 264000, Chin)

机构地区：[1]青岛大学医学院附属烟台毓璜顶医院泌尿外科,264000

出　　处：《临床外科杂志》2018年第1期51-53,共3页Journal of Clinical Surgery

摘　　要：目的分析Von Hippel-Lindau(VHL)综合征并发肾癌的临床特点,提高临床医生对VHL综合征的认识。方法回顾性分析我院泌尿外科收治的2例VHL综合征并发肾癌病人的临床资料,并结合文献复习。结果 2例中1例有家族史,行右肾肿物穿刺活检,病理未见癌细胞,病人后续就诊于其他医院,于当地医院外请专家行右肾癌根治术,术后病理检查提示肾透明细胞癌;1例无相关家族史,病人多发胰腺、肾脏囊肿,双肾多发肿物,肾肿物穿刺活检示肾透明细胞癌,无法行手术治疗,给予分子靶向药物舒尼替尼治疗2年,定期复查肾脏CT,发现肾肿物明显缩小。结论VHL综合征可发病于多个系统,其并发肾癌的特征不同于散发性肾癌。早期明确诊断和个体化治疗,能够提高VHL综合征病人生存率。Objective To investigate the clinical characteristics of renal cell carcinoma associated with von Hippel-Lindau（ VHL） syndrome and to improve the understanding on it. Methods Retrospective analysis combined with literature review was conducted on clinical data of two cases of VHL syndrome complicated with advanced renal cell carcinoma in urology of Yantai Yuhuangding Hospital. Results One case with family history was performed puncture biopsy at the right kidney. The pathology showed no significant malignant ingredients. The patient was further treated in the general hospital of Chinese People＇s Liberation Army and received laparoscopic radical nephrectom. The postoperative pathology showed clear cell carcinoma of the kidneys. Another patient without family history was diagnosed with pancreas and kidney cysts and multiple neoplasms in kidneys. Renal mass biopsy showed clear cell carcinoma,surgical treatment was excluded and molecular targeted drug sunitinib treatment was given for 2 years. The tumor size was significantly reduced during the periodical computed tomography（ CT） scan. Conclusion VHL syndrome occurs in multiple systems,the characteristics of its complicated renal cell carcinoma is different from that of sporadic renal carcinoma. Early diagnosis and personalized treatment can improve the survival rate of patients with VHL syndrome.

关 键 词：Von Hippel-Lindau综合征 肾细胞癌 手术治疗 靶向治疗 

分 类 号：R593.1[医药卫生—内科学] R737.11[医药卫生—临床医学]