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Birt-Hogg-Dubé Syndrome

Imaging findings

Chest CT shows multiple bilateral, flat, subpleural lung cysts. Abdominal CT demonstrates a synchronous chromophobe renal cell carcinoma.

Key takeaways

Birt-Hogg-Dubé syndrome is a rare genetic disorder characterized by lung cysts (with propensity for pneumothorax), fibrofolliculomas of the skin, and renal tumors (most commonly chromophobe RCC).

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