Original 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, W. Marston Linehan

Research output: Contribution to journalArticle

146 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)907-912
Number of pages6
JournalThe Journal of urology
Volume153
Issue number3 SUPPL.
DOIs
StatePublished - Jan 1 1995

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Kidney Neoplasms
Renal Cell Carcinoma
Clinical Studies
Penetrance
Neoplasms

All Science Journal Classification (ASJC) codes

  • Urology

Cite this

Zbar, B., Glenn, G., Lubensky, I., Choyke, P., Walther, M. M., Magnusson, G., ... Linehan, W. M. (1995). Original Articles: Kidney Cancer: Hereditary Papillary Renal Cell Carcinoma: Clinical Studies in 10 Families. The Journal of urology, 153(3 SUPPL.), 907-912. https://doi.org/10.1016/S0022-5347(01)67601-8

Original Articles : Kidney Cancer: Hereditary Papillary Renal Cell Carcinoma: Clinical Studies in 10 Families. / Zbar, Berton; Glenn, Gladys; Lubensky, Irina; Choyke, Peter; Walther, McClellan M.; Magnusson, Gosta; Bergerheim, Ulf S.R.; Pettersson, Silas; Amin, Mahul; Hurley, Kathy; Linehan, W. Marston.

In: The Journal of urology, Vol. 153, No. 3 SUPPL., 01.01.1995, p. 907-912.

Research output: Contribution to journalArticle

Zbar, B, Glenn, G, Lubensky, I, Choyke, P, Walther, MM, Magnusson, G, Bergerheim, USR, Pettersson, S, Amin, M, Hurley, K & Linehan, WM 1995, 'Original Articles: Kidney Cancer: Hereditary Papillary Renal Cell Carcinoma: Clinical Studies in 10 Families', The Journal of urology, vol. 153, no. 3 SUPPL., pp. 907-912. https://doi.org/10.1016/S0022-5347(01)67601-8
Zbar, Berton ; Glenn, Gladys ; Lubensky, Irina ; Choyke, Peter ; Walther, McClellan M. ; Magnusson, Gosta ; Bergerheim, Ulf S.R. ; Pettersson, Silas ; Amin, Mahul ; Hurley, Kathy ; Linehan, W. Marston. / Original Articles : Kidney Cancer: Hereditary Papillary Renal Cell Carcinoma: Clinical Studies in 10 Families. In: The Journal of urology. 1995 ; Vol. 153, No. 3 SUPPL. pp. 907-912.
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