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Risk of renal and colonic neoplasms and spontaneous pneumothorax in the Birt-Hogg-Dube syndrome

  1. Author:
    Zbar, B.
    Alvord, W. G.
    Glenn, G.
    Turner, M.
    Pavlovich, C. P.
    Schmidt, L.
    Walther, M.
    Choyke, P.
    Weirich, G.
    Hewitt, S. M.
    Duray, P.
    Gabril, F.
    Greenberg, C.
    Merino, M. J.
    Toro, J.
    Linehan, W. M.

  2. Author Address

    Natl Canc Inst Frederick, Lab Immunol, Ft Detrick, MD 21702 USA Natl Canc Inst Frederick, Lab Immunol, Ft Detrick, MD 21702 USA Natl Canc Inst Frederick, Lab Immunobiol, Ft Detrick, MD 21702 USA Natl Canc Inst Frederick, Data Management Serv, Ft Detrick, MD 21702 USA Natl Canc Inst Frederick, Intramural Res Support Program, Ft Detrick, MD 21702 USA Natl Canc Inst Frederick, Sci Applicat Int Corp, Ft Detrick, MD 21702 USA Natl Canc Inst, Dermatol Branch, Bethesda, MD 20894 USA Natl Canc Inst, Lab Pathol, Bethesda, MD 20894 USA Natl Canc Inst, Genet Epidemiol Branch, Div Canc Epidemiol & Genet, Bethesda, MD 20894 USA Natl Canc Inst, Ctr Canc Res, Bethesda, MD 20894 USA NIH, Clin Ctr, Bethesda, MD 20894 USA Natl Inst Diabet & Digest & Kidney Des, Digest Dis Branch, NIH, Bethesda, MD 20894 USA Univ Manitoba, Dept Pediat & Child Hlth, Winnipeg, MB, Canada Univ Manitoba, Dept Biochem & Med Genet, Winnipeg, MB, Canada Tech Univ Munich, D-81675 Munich, Germany Zbar B Natl Canc Inst Frederick, Lab Immunol, Ft Detrick, MD 21702 USA
    1. Year: 2002
  2. Journal: Cancer Epidemiology Biomarkers & Prevention
    1. 11
    2. 4
    3. Pages: 393-400
  4. Type of Article: Article
  1. Abstract:

    The Birt-Hogg-Dube syndrome, a genodermatosis characterized by benign tumors of the hair follicle, has been associated with renal and colonic neoplasms and spontaneous pneumothorax, but the risk of developing these disorders is unknown. We identified risk factors for renal tumors and spontaneous pneumothorax in 98 patients affected with the Birt-Hogg-Dub6 syndrome, in 13 Birt-Hogg-Dub6 haplotype carriers, and in 112 unaffected family members. Development of renal tumors was strongly associated with the Birt-Hogg-Dub6 syndrome and age. The odds ratio for renal tumor in BHD-affected family members adjusted for age was 6.9 (95% confidence interval, 1.5-31.6) and -9.0 for the other risk factors considered. Chromophobe renal carcinoma, an uncommon type of renal cancer, was the predominant type of renal cancer found. Spontaneous pneumothorax was also strongly associated with the Birt-Hogg- Dube syndrome and age. The odds ratio for pneumothorax in BHD- affected individuals, adjusted for age, was 50.3 (95% confidence interval, 6.4-392), and about 32 times higher adjusting for the other risk variables. Colon cancer and colon polyps were not related to the Birt-Hogg-Dub6 syndrome. The Birt-Hogg-Dub6 syndrome confers an increased risk for the development of renal tumors and spontaneous pneumothorax. We found no increase in risk for the development of colon polyps or colon carcinomas.

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