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An Unusual Case of Birt-Hogg-Dube Syndrome With Renal Involvement.

Janitzky Andreas نويسنده , Reiher Frank نويسنده , Porsch Markus نويسنده , Grube Christian نويسنده , Evert Matthias نويسنده , Liehr Uwe-Bernd نويسنده

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Recent investigations of the underlying pathophysiology of renal cell carcinoma (RCC) has resulted in the identification of involved molecular pathways, including the inactivation of the von Hippel-Lindau gene in most sporadic cases of RCC.(1) They are characterized by one specific histological type. In contrast, kidney tumors in patients with Birt-Hogg-Dube syndrome (BHD) consist of a variety of histological types.(2) We report a patient with multiple renal cell tumors in one kidney with a history of spontaneous pneumothorax, but without skin lesions which are typical signs of BHD syndrome.

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