Parotid Acinic Cell Carcinoma as a Presentation of Birt-Hogg-Dube Syndrome.

Birt-Hogg-Dube syndrome (BHD) is a rare autosomal dominant disease classically associated with fibrofolliculomas, pulmonary cysts, spontaneous pneumothorax, and renal cancers. Information about its manifestation aside from the ones listed prior is limited. There have been several reports of BHD associated with parotid oncocytomas and rare benign epithelial tumors. Here, we report the first known case of BHD in association with parotid acinic cell carcinoma, a rare low-grade malignant tumor of salivary glands.