Renal cell neoplasms in Birt-Hogg-Dubé (BHD) syndrome


  • An autosomal dominant syndrome characterized by a constellation of benign skin follicle tumors, multiple renal cell neoplasms, and spontaneous pneumothorax

Clinical features


  • Rare autosomal dominant disease with an incidence of 1/36,000 to 1/45,000

  • Life risk for renal cell carcinomas (RCCs) 14%–35%

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