von-Hippel-Lindau综合征并发肾癌二例报告

目的探讨von-Hippel-Lindau（VHL）综合征并发肾癌的临床诊断和治疗水平。方法回顾分析2008年9月2008年10月分别收治的2例VHL综合征并发双肾多发肿瘤患者的临床诊治过程并结合文献进行讨论。结果 2例患者均为女性。例1为36岁,因腰痛不适3个月入院。既往8年有左眼视网膜血管母细胞瘤手术史。入院后双肾CT检查发现双肾肿瘤伴囊肿,考虑诊断为VHL综合征并发双侧肾癌。患者因故拒绝手术治疗,随访至今,患者病情无明显变化。例2为41岁,因B型超声发现右肾占位4年,反复肉眼血尿2年入院。4年前行小脑蚓部血管母细胞瘤切除术。入院后CT检查显示右肾巨大实性肿瘤伴多发囊肿,左肾多发性小的囊实性占位;胰腺多发小囊肿。诊断为VHL综合征并发右侧肾癌,行右肾根治性切除。手术后病理检查为右肾透明细胞癌,手术后随访至今病情稳定。结论 VHL综合征并发肾癌临床少见,若患者表现为双侧、多发性、囊实性肾肿瘤,应考虑VHL综合征并发肾癌。及时正确诊断并根据患者病情制定个体化治疗方案,尽量避免肾切除,保留肾单位,手术结合影像学观察是主要治疗策略。

von-Hippel-Lindau Syndrome：Two Cases Report and Review of the Literature

Objective To investigate the diagnosis and treatment of renal tumor in von-Hippel-Lindau（VHL） disease. Methods The clinical data of two patients of VHL disease with renal tumor were retrospectively analyzed and review of literatures. Results Two patients were female. The first patient with 36 years old who was admitted because of lumbar pain for three months,and had operation for retinal hemangioblastoma of left eye eight years before. CT indicated bilateral renal tumor and renal cysts. VHL syndrome with bilateral renal carcinoma was diagnosed and the patient refused operation.and the patient is stable with little changes througth follow-up. The second patient with 41 years old who was admitted because of right renal tumor diagnosed by ultrasonic B for four years and intermittent haematuria for two years. The patient had operation for cerebellar hemangioblastoma four years before. CT indicated right huge renal tumor with mutiple renal cysts.,and multiple left renal tumor and cysts. VHL syndrome with right renal carcinoma was diagnosed and radical nephrectomy was followed ,and the pathology was renal clear carcinoma.and the patient is stable with little changes througth follow-up. Conclusion VHL syndrome should be considered when renal tumor with bilateral,multicentric or with multiple cysts. Treatment should be individualized with watchful waiting with nephron-sparing surgery. Nephrectomy should be avoided when possible.