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. 2009 Dec;24(6):1145-9.
doi: 10.3346/jkms.2009.24.6.1145. Epub 2009 Nov 9.

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

Won Tae Kim  1 , Won Sik Ham, Hee Jeong Ju, Jin Sun Lee, Jin Sung Lee, Young Deuk Choi
Affiliations

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

Won Tae Kim et al. J Korean Med Sci. 2009 Dec.

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.

Keywords: Carcinoma, Renal Cell; Incidence; Von Hippel-Lindau Disease.

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Figures

Fig. 1
Fig. 1
Overall disease-specific survival rates for patients with von Hippel-Lindau (VHL) disease only, and VHL with and without renal cell carcinoma (RCC).
Fig. 2
Fig. 2
Cancer-specific survival rates of patients with both von Hippel- Lindau (VHL) disease and RCC, versus patients with sporadic bilateral or multifocal RCC.
See this image and copyright information in PMC

References

    1. Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM, Oldfield EH. von Hippel-Lindau disease. Lancet. 2003;361:2059–2067. - PubMed
    1. Latif F, Tory K, Gnarra J, Yao M, Duh FM, Orcutt ML, Stackhouse T, Kuzmin I, Modi W, Geil L. Identification of the von Hippel-Lindau disease tumour suppressor gene. Science. 1993;260:1317–1320. - PubMed
    1. Maher ER, Iselius L, Yates JR, Littler M, Benjamin C, Harris R, Sampson J, Williams A, Ferguson-Smith MA, Morton N. Von Hippel-Lindau disease: a genetic study. J Med Genet. 1991;28:443–447. - PMC - PubMed
    1. Kim DS, Kim JJ, Yoon DK, Koh SK, Won NH. A case of von Hippel-Lindau disease diagnosed by initial symptoms of urologic manifestation. Korean J Urol. 1987;28:126–132.
    1. Kang IS, Lee YI, Seo IY, Jeong HJ, Rim JS. Renal cell carcinoma associated with von Hippel-Lindau disease treated by wedge resection. Korean J Urol. 2004;45:495–497.

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