Tuberous sclerosis complex with angiomyolipomatosis

Case contributed by Dr Joe Mullineux

Presentation

Known tuberous sclerosis presents with frank hematuria.

Patient Data

Age: 70 years
Gender: Female

Due to history of hematuria a bleeding protocol CT was performed.

The pre-contrast study shows bilateral multiple large fat containing renal masses in keeping with bilateral angiomyolipomatosis causing significant mass effect. There are three sites of previous vascular embolization coils in the right kidney (upper and lower pole). There are also small foci of calcification in some of the angiomyolipomas (AMLs).

On lung windows the patient has multiple thin-walled cysts at both lung bases in keeping with TS complex. There is some chronic associated interlobular septal thickening, patchy consolidation and atelectasis. Findings are consistent with tuberous sclerosis complex (TSC) associated lymphangioleiomyomatosis (LAM).

Ring pessary noted within the vagina.

No active contrast extravasation was demonstrated on arterial phase imaging.

Delayed imaging shows no contrast pooling. This phase also delineates the collecting system and residual renal parenchyma nicely.

Case Discussion

This case is a nice illustration of TSC with lung and renal disease. It also highlights the association between TS and multiple bilateral large renal angiomyolipomas and potential complications. The propensity to bleed increases with the size of AMLs which tend to be bigger in patients with TS1. The multiplicity of lesions also increases risk of bleeding for obvious reasons. As we can see this patient has undergone multiple embolization procedures previously. On this study there was no active hemorrhage. 

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