Abstract | PURPOSE: MATERIALS AND METHODS: We established registers comprising 63 subjects with von Hippel-Lindau renal cell carcinoma, belonging to 30 distinct families (register A), and 460 unselected patients operated on for renal cell carcinoma in an 11-year period (register B). Molecular genetic analysis of the von Hippel-Lindau gene was performed for living patients of register A, representing 80% of von Hippel-Lindau families, and register B, 62% living patients, to identify von Hippel-Lindau germline mutations. In addition, register B was evaluated by a questionnaire (95% response) for familial occurrence of von Hippel-Lindau disease. RESULTS: CONCLUSIONS: von Hippel-Lindau differs from sporadic renal cell carcinoma in morphology and biology. Our data provide arguments for planning surgery for von Hippel-Lindau renal cell carcinoma and should stimulate future investigations.
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Authors | H P Neumann, B U Bender, D P Berger, J Laubenberger, W Schultze-Seemann, U Wetterauer, F J Ferstl, E W Herbst, G Schwarzkopf, F J Hes, C J Lips, J M Lamiell, O Masek, P Riegler, B Mueller, D Glavac, H Brauch |
Journal | The Journal of urology
(J Urol)
Vol. 160
Issue 4
Pg. 1248-54
(Oct 1998)
ISSN: 0022-5347 [Print] United States |
PMID | 9751329
(Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)
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Topics |
- Adult
- Aged
- Aged, 80 and over
- Carcinoma, Renal Cell
(complications, epidemiology, genetics, pathology)
- Female
- Humans
- Kidney Neoplasms
(complications, epidemiology, genetics, pathology)
- Male
- Middle Aged
- Mutation
- Prevalence
- von Hippel-Lindau Disease
(complications, epidemiology, genetics)
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