Birt-Hogg-Dubé syndrome presenting with pneumothorax
Imaging findings
CT shows a small, asymptomatic spontaneous pneumothorax and multiple thin-walled, basally-predominant, perivenous lung cysts. Abdominal CT demonstrates a slowly growing, slowly enhancing solid renal mass.
Key takeaways
Birt-Hogg-Dubé syndrome is an autosomal dominant condition characterized by basally predominant lung cysts, spontaneous pneumothorax, and renal neoplasms (most commonly chromophobe renal cell carcinoma or oncocytoma).
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