Von-Hippel-Lindau病并发肾癌2例

doi:10.6040/j.issn.1671-7554.0.2015.748

摘要/Abstract

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。

关键词: 肾细胞肿瘤, Von-Hippel-Lindau病, 家族性遗传病, 临床特征

Abstract: Objective To explore the clinical characteristics of Von Hippel-Lindau(VHL)disease complicated with renal carcinoma, in order to find better methods of diagnosis and treatment. Methods Clinical data of 2 cases of VHL disease complicated with renal carcinoma were retrospectively analyzed and relevant literature was reviewed. Results One of the 2 patients had family history and underwent right radical nephrectomy. Pathological examination suggested clear-cell carcinoma. No recurrence was found in this patient after one-year follow-up. The other patient did not have family history. He also underwent right radical nephrectomy. Pathological examination suggested multilocular cystic renal cell carcinoma in the right kidney and pheochromocytoma in the right adrenal gland. Four years later, carcinoma was found in the left kidney and left adrenal gland. Pathological examination of biopsy tissue in the left kidney tumor indicated clear-cell carcinoma. He received sorafenib, a molecular targeted drug, and regular hemodialysis. Conclusion VHL disease is a rare cancer syndrome that involves multiple systems. The characteristics of renal cancer concurrent with VHL are different from sporadic one. Early diagnosis and individualized treatment can improve the quality of patients' life. New treatments should be further explored.

Key words: Von Hippel-Lindau disease, Renal tumor, Familial hereditary disease, Clinical characteristics

中图分类号:

R596.1

王光杰,俎树禄,张秀琳,徐祗顺,周春文,刘玉强,王绍勇. Von-Hippel-Lindau病并发肾癌2例[J]. 山东大学学报（医学版）, 2016, 54(2): 86-89.

WANG Guangjie, ZU Shulu, ZHANG Xiulin, XU Zhishun, ZHOU Chunwen, LIU Yuqiang, WANG Shaoyong. Von Hippel-Lindau disease complicated with renal carcinoma: a report of 2 cases[J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2016, 54(2): 86-89.

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