Radiological evaluation, management, and surveillance of renal masses in Von Hippel-Lindau disease.
Von Hippel-Lindau disease (VHL) is a rare, autosomal-dominant condition that predisposes patients to developing renal cysts and tumours. VHL is the most common of the hereditary renal cancer syndromes, and is exclusively associated with the clear cell histological subtype of renal cell carcinoma. This review focuses on the genetics, surveillance, and management of complex renal masses in VHL. The current status of renal cyst analysis in general and in the context of VHL is also reviewed.
Department of Radiology, St Georges Hospital, London, UK.
M
M
Meister
P
P
Choyke
C
C
Anderson
U
U
Patel
D000328:Adult; D002292:Carcinoma, Renal Cell / Q000175:diagnosis* / Q000209:etiology / Q000235:genetics; D003287:Contrast Media; D003560:Cysts / Q000175:diagnosis / Q000209:etiology / Q000235:genetics; D006801:Humans; D007674:Kidney Diseases / Q000150:complications / Q000235:genetics; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000235:genetics; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed / Q000379:methods; D044767:Ubiquitin-Protein Ligases / Q000235:genetics; D014463:Ultrasonography; D051794:Von Hippel-Lindau Tumor Suppressor Protein; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000235:genetics
D003287:Contrast Media; D044767:Ubiquitin-Protein Ligases; D051794:Von Hippel-Lindau Tumor Suppressor Protein; C497446:VHL protein, human