CTICasesTuberous Sclerosis Complex with Lymphangioleiomyomatosis
Imaging findings
Chest CT demonstrates innumerable thin-walled, well-defined, round lung cysts distributed uniformly throughout both lungs, classic for lymphangioleiomyomatosis. Abdominal CT demonstrates massive, extensive angiomyolipomas (AMLs) replacing a significant portion of both the kidneys and the liver. Additionally, a small, fatty myocardial focus is noted.
Key takeaways
Tuberous sclerosis complex is a genetic disorder characterized by multisystem hamartomas, including renal and hepatic angiomyolipomas, cardiac rhabdomyomas, and pulmonary lymphangioleiomyomatosis (LAM). Intra-myocardial fatty foci are highly specific markers of tuberous sclerosis complex and can help differentiate it from sporadic LAM.
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