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Renal cell neoplasms in Birt-Hogg-Dubé (BHD) syndrome
Definition
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An autosomal dominant syndrome characterized by a constellation of benign skin follicle tumors, multiple renal cell neoplasms, and spontaneous pneumothorax
Clinical features
Epidemiology
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Rare autosomal dominant disease with an incidence of 1/36,000 to 1/45,000
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Life risk for renal cell carcinomas (RCCs) 14%–35%
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