Oncocytic carcinoma of the parotid gland as a manifestation of Birt-Hogg-Dube syndrome

Renal oncocytomas are commonly reported in association with Birt-Hogg-Dube (BHS) syndrome, while BHD-associated oncocytomas of the parotid gland are rare. To date, there have been only 11 cases of BHD-associated parotid gland oncocytoma, without a reported case of malignant transformation. We present the first reported case of oncocytic carcinoma of the parotid gland associated with BHD, with radiologic and histologic correlation. This case establishes that BHD-associated parotid oncocytic lesions, previously identified only as benign oncocytomas in the literature to date, can undergo malignant transformation, and should potentially be regarded with a higher index of suspicion and lower threshold for aggressive management.