Table 2. Kidney-related surveillance and management recommendations [9, 13].

Newly diagnosed or suspected	TSC diagnosed with definite or possible TSC
Surveillance of kidneys
Obtain MRI of the abdomen to assess for the presence of angiomyolipoma and renal cysts. Screen for hypertension by obtaining accurate blood pressure. Evaluate renal function by determining GFR.	Obtain MRI of the abdomen to assess angiomyolipoma progression and renal cystic disease (every 1 to 3 years, life-long). Assess renal function (GFR and blood pressure) at least annually.
Clinical presentation	Recommendation
Management recommendations for renal angiomyolipoma
Angiomyolipoma with acute hemorrhage	Embolization (followed by corticosteroids for 7 days to mitigate postembolization syndrome) [14]. Embolization should be as selective as technically feasible to preserve renal parenchyma. Avoid nephrectomy.
Asymptomatic, growing angiomyolipoma > 3 cm in diameter.	First-line: mTOR inhibitor. Second-line: selective embolization or kidney-sparing resection.

GFR = glomerular filtration rate; MRI = magnetic resonance imaging; mTOR = mammalian target of rapamycin; TSC = tuberous sclerosis complex.