Clinical characteristics of renal cell carcinoma in Korean patients with von Hippel-Lindau disease compared to sporadic bilateral or multifocal renal cell carcinoma.

Journal of Korean Medical Science
Won Tae KimYoung Deuk Choi

Abstract

This study was done to analyze the clinical characteristics of renal cell carcinoma (RCC) in Korean patients with von Hippel-Lindau (VHL) disease. Between January 1996 and July 2008, 1,514 patients were diagnosed with RCC and 24 patients were diagnosed with VHL disease at our institute. We analyzed the clinical characteristics of the 24 patients diagnosed with VHL. The mean age of patients with VHL was 39.2+/-12.6 yr; the mean age of patients with both VHL and RCC was 42.5+/-10.3 yr. Among the 24 patients with VHL, 7 patients had retinal angiomas, 11 had RCC, 16 had renal lesions, 18 had pancreatic lesions and 21 had cerebellar hemangioblastomas. There was no significant difference between survival rates of patients with VHL alone and those with VHL and RCC. However, cancer-specific survival rates were significantly different between patients with both VHL and RCC and patients with sporadic bilateral or multifocal RCC. In our Korean study, the incidence of RCC in patients with VHL disease is 45.8% and the incidence of VHL disease in patients with RCC is 0.73%. Due to the low overall incidence of VHL in Korea, extended multi-institutional studies are needed to establish the true characteristics of VHL disease.

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Citations

Jan 20, 2012·The Journal of Clinical Endocrinology and Metabolism·Allison B WeisbrodElectron Kebebew
Mar 29, 2014·Journal of Cancer Research and Clinical Oncology·Taekmin KwonChoung-Soo Kim
Apr 6, 2016·Frontiers in Oncology·John Clark Henegan, Christian R Gomez
Oct 30, 2019·Orphanet Journal of Rare Diseases·Patrick SchuhmacherStefan Zschiedrich

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Methods Mentioned

BETA
biopsies

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