Diagnosis and treatment of renal lesions in 1 0 patients with Von Hippel-Lindau syndrome
Dong Yi
Ju Guanqun
Wu Zhenjie
Liu Tao
Chen Jie
He Yi
Liu Bing
Wang Linhui
Abstract：Objective:To investigate the clinical features,diagnosis and management of renal lesions in pa-tients 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 pa-tients 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 syn-drome 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 le-sions.The optimal individualized treatment should under Multidisciplinary team (MDT),according to the general condition of patient,in order to improve prognosis.
Keywords：von Hippel-Lindau syndromerenal lesionrenal cell carcinomarenal cyst
Publication Date：2018-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：5( 196-200 )