What are familial renal oncocytoma and Birt-Hogg-Dube syndrome?

Updated: May 21, 2019
  • Author: Kush Sachdeva, MD; Chief Editor: E Jason Abel, MD  more...
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Answer

Individuals affected with familial renal oncocytoma can develop bilateral, multifocal oncocytoma or oncocytic neoplasms in the kidney. Birt-Hogg-Dube syndrome is a hereditary cutaneous syndrome. Patients with Birt-Hogg-Dube syndrome have a dominantly inherited predisposition to develop benign tumors of the hair follicle (ie, fibrofolliculomas), predominantly on the face, neck, and upper trunk, and these individuals are at risk of developing renal tumors, colonic polyps or tumors, and pulmonary cysts.


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