CTICasesTuberous Sclerosis with Lymphangioleiomyomatosis, Myocardial Fatty Lesion, and Mediastinal Mass
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Imaging findings
Chest CT shows diffuse, thin-walled, round cystic changes throughout both lungs consistent with lymphangioleiomyomatosis (LAM), absent kidneys (previously resected), multiple hepatic lesions, a fatty unencapsulated lesion in the myocardium, and a large fat-containing mediastinal mass centered in the anterior/middle mediastinum resembling an angiomyolipoma.
Key takeaways
Tuberous sclerosis complex is a multisystem neurocutaneous disorder characterized by benign hamartomatous lesions, including cardiac rhabdomyomas (which appear as myocardial fatty lesions), renal and hepatic angiomyolipomas, lymphangioleiomyomatosis (LAM), and rare fat-containing mediastinal masses such as mediastinal angiomyolipomas or lipomas.
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