Article
Pulmonary cysts of Birt-Hogg-Dubé syndrome: a clinicopathologic and immunohistochemical study of 9 families.
The American journal of surgical pathology - 1 Apr 2012
Furuya Mitsuko, Tanaka Reiko, Koga Shunsuke, Yatabe Yasushi, Gotoda Hiroko, Takagi Seiji, Hsu Yung-Hsiang, Fujii Takeshi, Okada Akira, Kuroda Naoto, Moritani Suzuko, Mizuno Hideki, Nagashima Yoji, Nagahama Kiyotaka, Hiroshima Kenzo, Yoshino Ichiro, Nomura Fumio, Aoki Ichiro, Nakatani Yukio
Abstract excerpt
Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant disorder characterized by fibrofolliculomas, renal tumors, and pulmonary cysts with recurrent pneumothorax. Multiple pulmonary cysts and pneumothorax are the key signs for diagnosing BHD syndrome. The pathologic features of BHD pulmonary cysts, however, are poorly understood. This disorder is caused by mutations in the gene that encodes folliculin (FLCN)....
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