CTICasesLymphangioleiomyomatosis and Renal Angiomyolipomas in Tuberous Sclerosis Complex
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Imaging findings
Abdominal computed tomography demonstrates large bilateral renal angiomyolipomas containing macroscopic fat, with a solid, enhancing clear cell renal cell carcinoma component on the left side. High-resolution chest computed tomography reveals numerous thin-walled, diffuse, bilateral pulmonary cysts distributed uniformly throughout the lung parenchyma, including the lung bases. Additionally, a broad-based, low-attenuation fatty lesion is identified along the interventricular septum of the heart.
Key takeaways
Lymphangioleiomyomatosis is strongly associated with tuberous sclerosis complex, an autosomal dominant neurocutaneous syndrome. Unlike sporadic lymphangioleiomyomatosis which typically presents with isolated pulmonary cysts, patients with tuberous sclerosis complex commonly present with bilateral renal angiomyolipomas, cardiac rhabdomyomas or lipomas, and a higher overall risk of developing pulmonary cystic disease.
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