CTICasesBirt-Hogg-Dubé Syndrome with Adrenal Oncocytoma
Imaging findings
Abdominal CT shows a large, hemorrhagic left adrenal mass with high internal attenuation. Chest CT demonstrates a small number of thin-walled, subpleural, and lenticular pulmonary cysts clustered in the lower lobes. Genetic testing confirmed a mutation in the folliculin gene. Pathology of the resected mass showed an oncocytic adrenocortical tumor.
Key takeaways
Birt-Hogg-Dubé syndrome is an autosomal dominant condition characterized by benign skin tumors (fibrofolliculomas), lung cysts with a predisposition to spontaneous pneumothoraces, and renal tumors. Oncocytic adrenocortical tumors represent a rare, atypical neoplastic presentation associated with this syndrome.
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