Perinephric angiomyolipomas in tuberous sclerosis

Abstract

Bilateral large perinephric angiomyolipomas in a young Chinese woman with tuberous sclerosis are reported. Computed tomography (CT) demonstrated large fatty masses, containing abnormally dilated blood vessels and muscle strands, within the Gerota's fascia bilaterally. There were multiple foci of involvement in both kidneys. The diagnosis was confirmed on histological examination of the excised left perinephric angiomyolipoma and nephrectomy specimens. The patient subsequently developed bilateral pneumothoraces which were surgically treated. High resolution CT showed extensive thin-walled cysts in both lungs.