Tuberous sclerosis complex with lymphangioleiomyomatosis (LAM) and renal angiomyolipomas
Presentation
Underlying tuberous sclerosis. Left nephrectomy done due to renal cell carcinoma.
Patient Data
Diffuse small thin-walled cystic lesions scattered in both lungs fields consistent with lymphangioleimyomatosis. No pneumothorax or pleural effusion.
These are better seen on the thin-sliced images.
Right kidney is enlarged with multiple heterogeneous fat density lesions replacing most the normal renal parenchyma in keeping with angiomyolipomas. No hydronephrosis or hydroureter. Left kidney is absent in keeping with previous nephrectomy.
Case Discussion
Tuberous sclerosis (TS) is a phakomatosis involving many organ systems.
Lymphangioleiomyomatosis is almost always seen in women and may be associated with tuberous sclerosis complex. In this patient, the size of the lung cysts are small, which may be due to early disease. These cysts may progressively enlarge and increase in number over time. Pneumothorax is common complication, however is not seen in this patient.
Renal angiomyolipomas and renal cysts are common renal manifestation of TS. Rarely, they may also develope renal cell carcinomas and oncocytomas. Although the incidence of renal cell carcinoma is not increased as compared to the general populations, RCC tends to occur earlier (at a younger age) with slower growth in patients with tuberous sclerosis 1.
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