CTICasesTuberous Sclerosis with Cardiac Lipomas, Renal/Hepatic Angiomyolipomas, and Pulmonary LAM
Imaging findings
In a patient with known tuberous sclerosis and multiple renal angiomyolipomas (status post right nephrectomy) and hepatic fatty lesions, non-contrast CT additionally shows numerous small fatty deposits scattered throughout the myocardium -- more numerous than the one or two lesions typically seen with tuberous-sclerosis-associated cardiac lipomas -- along with several sclerotic bone islands and a scattering of thin-walled pulmonary cysts.
Key takeaways
Tuberous sclerosis can produce an unusually high burden of small cardiac lipomatous deposits beyond the classically described solitary or paucifocal lesions, and the presence of multiple sclerotic bone islands (more than four or five) is a described discriminator favoring tuberous sclerosis over sporadic LAM when pulmonary cysts are also present; pulmonary LAM occurs in a minority of male tuberous sclerosis patients and is usually mild, as in this incidentally noted case.
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