Management of Renal Angiomyolipomas in Tuberous Sclerosis: A Case Series.

Abstract

Renal angiomyolipomas, benign tumors composed of blood vessels, adipose tissue, and smooth muscle, affect approximately 70% to 80% of patients with tuberous sclerosis. Angiomyolipomas smaller than 4 cm are usually asymptomatic, whereas larger ones can cause lumbar pain, anemia, and hematuria. Contrary to its sporadic counterparts, tuberous sclerosis-associated angiomyolipomas often present at a young age, are multicentric and large, and carry a higher risk of life-threatening hemorrhage. Therapeutic strategies include selective tumor embolization, nephrectomy for severe cases, and medical treatment such as everolimus. Despite a correlation between tuberous sclerosis and renal angiomyolipomas and their described high complication rates in literature, disease awareness in clinical practice remains low. This case series describes the management and outcomes of three patients with tuberous sclerosis-associated renal angiomyolipomas treated with selective arterial embolization.