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- W2069899769 abstract "No AccessJournal of UrologyClinical Urology: Original Article1 Mar 1995Original Articles: Kidney Cancer: Hereditary Papillary Renal Cell Carcinoma: Clinical Studies in 10 Families Berton Zbar, Gladys Glenn, Irina Lubensky, Peter Choyke, McClellan M. Walther, Gosta Magnusson, Ulf S.R. Bergerheim, Silas Pettersson, Mahul Amin, Kathy Hurley, and W. Marston. Linehan Berton ZbarBerton Zbar More articles by this author , Gladys GlennGladys Glenn More articles by this author , Irina LubenskyIrina Lubensky More articles by this author , Peter ChoykePeter Choyke More articles by this author , McClellan M. WaltherMcClellan M. Walther More articles by this author , Gosta MagnussonGosta Magnusson More articles by this author , Ulf S.R. BergerheimUlf S.R. Bergerheim More articles by this author , Silas PetterssonSilas Pettersson More articles by this author , Mahul AminMahul Amin More articles by this author , Kathy HurleyKathy Hurley More articles by this author , and W. Marston. LinehanW. Marston. Linehan More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(01)67601-8AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail We recently described a 3-generation family with members affected with papillary renal cell carcinoma, an uncommon histological type of renal cell carcinoma. Possibly family 150 is an isolated occurrence, a reflection of some as yet unknown environmental factor. Alternatively, family 150 may represent a distinct class of inherited cancer. To distinguish between these 2 possibilities we sought additional families with papillary renal cell carcinoma and we identified 9 with members affected with papillary renal cell carcinoma. There were 29 affected male and 12 affected female subjects (ratio 2.41:1), including affected members of family 150. Papillary renal cell carcinomas were often detected incidentally in asymptomatic individuals or during screening of asymptomatic members of renal cell carcinoma families. The penetrance, the proportion of obligate gene carriers that showed clinical evidence of the disease, was reduced. The median survival of affected individuals was 52 years. The results support the concept that the predisposition to develop papillary renal cell carcinomas may be inherited and that hereditary papillary renal cell carcinoma constitutes a distinct class of inherited cancer. References 1 : von Hippel-Lindau disease affecting 43 members of a single kindred. Medicine1989; 68: 1. Google Scholar 2 : Identification of the von Hippel-Lindau disease tumor suppressor gene. Science1993; 260: 1317. Crossref, Medline, Google Scholar 3 : VHL tumor suppressor gene mutations in renal carcinoma tumorigenesis. Nature Gen.1994; 7: 85. 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Google Scholar From the Laboratories of Immunobiology and Pathology, Surgery Branch and Cancer Diagnosis Branch, National Cancer Institute, Department of Diagnostic Radiology, National Institutes of Health, Bethesda, Maryland, Department of Renal Medicine, Huddinge Hospital, Huddinge, Department of Surgery, Karolinska Hospital, Stockholm, Department of Urology, Sahlgrenska Hospital, Gothenburg, Sweden, and Department of Pathology, Henry Ford Hospital, Detroit, Michigan© 1995 by American Urological Association, Inc.FiguresReferencesRelatedDetailsCited byLaguna M (2016) Re: The Subclassification of Papillary Renal Cell Carcinoma Does Not Affect Oncological Outcomes after Nephron Sparing SurgeryJournal of Urology, VOL. 196, NO. 3, (686-687), Online publication date: 1-Sep-2016.SCHMIDT L, NICKERSON M, ANGELONI D, GLENN G, WALTHER M, ALBERT P, WARREN M, CHOYKE P, TORRES-CABALA C, MERINO M, BRUNET J, BÉREZ V, BORRÀS J, SESIA G, MIDDELTON L, PHILLIPS J, STOLLE C, ZBAR B, PAUTLER S and LINEHAN W (2018) EARLY ONSET HEREDITARY PAPILLARY RENAL CARCINOMA: GERMLINE MISSENSE MUTATIONS IN THE TYROSINE KINASE DOMAIN OF THE MET PROTO-ONCOGENEJournal of Urology, VOL. 172, NO. 4 Part 1, (1256-1261), Online publication date: 1-Oct-2004.DRACHENBERG D, MENA O, CHOYKE P, LINEHAN W and WALTHER M (2018) PARENCHYMAL SPARING SURGERY FOR CENTRAL RENAL TUMORS IN PATIENTS WITH HEREDITARY RENAL CANCERSJournal of Urology, VOL. 172, NO. 1, (49-53), Online publication date: 1-Jul-2004.LINEHAN W, WALTHER M and ZBAR B (2018) The Genetic Basis of Cancer of the KidneyJournal of Urology, VOL. 170, NO. 6, (2163-2172), Online publication date: 1-Dec-2003.HERRING J, ENQUIST E, CHERNOFF A, LINEHAN W, CHOYKE P and WALTHER M (2018) PARENCHYMAL SPARING SURGERY IN PATIENTS WITH HEREDITARY RENAL CELL CARCINOMA: 10-YEAR EXPERIENCEJournal of Urology, VOL. 165, NO. 3, (777-781), Online publication date: 1-Mar-2001.TAKAKI Y, FURIHATA M, YOSHIKAWA C, KISHIDA T, YAO M and SHUIN T (2018) SPORADIC BILATERAL PAPILLARY RENAL CARCINOMA EXHIBITING C-MET MUTATION IN THE LEFT KIDNEY TUMORJournal of Urology, VOL. 163, NO. 4, (1241-1242), Online publication date: 1-Apr-2000.ORNSTEIN D, LUBENSKY I, VENZON D, ZBAR B, LINEHAN W and WALTHER M (2018) PREVALENCE OF MICROSCOPIC TUMORS IN NORMAL APPEARING RENAL PARENCHYMA OF PATIENTS WITH HEREDITARY PAPILLARY RENAL CANCERJournal of Urology, VOL. 163, NO. 2, (431-433), Online publication date: 1-Feb-2000.ZAMBRANO N, LUBENSKY I, MERINO M, LINEHAN W and WALTHER M (2018) HISTOPATHOLOGY AND MOLECULAR GENETICS OF RENAL TUMORS: TOWARD UNIFICATION OF A CLASSIFICATION SYSTEMJournal of Urology, VOL. 162, NO. 4, (1246-1258), Online publication date: 1-Oct-1999.WALTHER M, CHOYKE P, GLENN G, LYNE J, RAYFORD W, VENZON D and LINEHAN W (2018) RENAL CANCER IN FAMILIES WITH HEREDITARY RENAL CANCER: PROSPECTIVE ANALYSIS OF A TUMOR SIZE THRESHOLD FOR RENAL PARENCHYMAL SPARING SURGERYJournal of Urology, VOL. 161, NO. 5, (1475-1479), Online publication date: 1-May-1999. Volume 153Issue 3SMarch 1995Page: 907-912 Advertisement Copyright & Permissions© 1995 by American Urological Association, Inc.MetricsAuthor Information Berton Zbar More articles by this author Gladys Glenn More articles by this author Irina Lubensky More articles by this author Peter Choyke More articles by this author McClellan M. Walther More articles by this author Gosta Magnusson More articles by this author Ulf S.R. Bergerheim More articles by this author Silas Pettersson More articles by this author Mahul Amin More articles by this author Kathy Hurley More articles by this author W. Marston. Linehan More articles by this author Expand All Advertisement PDF downloadLoading ..." @default.
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