CTICasesLymphangioleiomyomatosis
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Imaging findings
CT imaging demonstrates a right-sided pneumothorax and bilateral, thin-walled, round cysts uniformly scattered throughout both lungs. Abdominal imaging reveals fatty lesions in the liver and kidneys, classic for angiomyolipomas.
Key takeaways
Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease characterized by the clonal proliferation of abnormal smooth muscle-like cells (LAM cells) along airways, blood vessels, and lymphatics, leading to airway obstruction and cyst formation. It occurs almost exclusively in women, is associated with tuberous sclerosis complex (TSC), and frequently presents with recurrent pneumothoraces and abdominal angiomyolipomas.
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