Abstract：Objective: To investigate the clinical features, diagnosis and management of renal lesions in patients with von Hippel-Lindau (VHL) syndrome and improve the current understanding on renal lesions. Methods: The clinical data of ten VHL syndrome patients with renal lesions were analyzed retrospectively, including clinical features, laboratory findings, imaging, pathological features, operation method and follow-up. Results: Eight patients with multiple clear cell renal cell carcinoma, six patients had renal cystic lesions, eight patient had retinal multiple angioma or angioma in central neural system (CNS), and two had family history of hemangioblastoma in CNS. All the ten patients had stable disease status, and followed up by outpatient visits. Conclusions: VHL syndrome can manifest as renal cell carcinoma and renal cystic lesions. Treatment contain active surveillance, surgical intervention and medical treatment. Nowadays there is no guideline specifically focused on VHL disease renal lesions. The optimal individualized treatment should under Multidisciplinary team (MDT), according to the general condition of patient, in order to improve prognosis.
董毅, 琚官群, 吴震杰, 刘涛, 陈杰, 何屹, 刘冰, 王林辉. Von Hippel-Lindau综合征肾脏病变10例诊治分析[J]. 微创泌尿外科杂志, 2018, 7(3): 196-200.
Dong Yi, Ju Guanqun, Wu Zhenjie, Liu Tao, Chen Jie, He Yi, Liu Bing, Wang Linhui. Diagnosis and treatment of renal lesions in 10 patients with Von Hippel-Lindau syndrome. JOURNAL OF MINIMALLY INVASIVE UROLOGY, 2018, 7(3): 196-200.
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