Renal tumors in the Birt-Hogg-Dube syndrome
CP Pavlovich, MCM Walther, RA Eyler… - The American journal …, 2002 - journals.lww.com
Abstract Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant genodermatosis
characterized by the development of small dome-shaped papules on the face, neck, and
upper trunk (fibrofolliculomas). In addition to these benign hair follicle tumors, BHD confers
an increased risk of renal neoplasia and spontaneous pneumothorax. To date, there has
been no systematic pathologic analysis of the renal tumors associated with this syndrome.
We reviewed 130 solid renal tumors resected from 30 patients with BHD in 19 different …
characterized by the development of small dome-shaped papules on the face, neck, and
upper trunk (fibrofolliculomas). In addition to these benign hair follicle tumors, BHD confers
an increased risk of renal neoplasia and spontaneous pneumothorax. To date, there has
been no systematic pathologic analysis of the renal tumors associated with this syndrome.
We reviewed 130 solid renal tumors resected from 30 patients with BHD in 19 different …