Guideline sources The following summarized guidelines for the evaluation and management of renal angiomyolipoma are prepared by our editorial team based on guidelines from the Tuberous Sclerosis Complex International (TSCi 2013) and the European Respiratory Society (ERS 2010). 1 2 Guidelines 1. Diagnostic investigations Diagnostic imaging: As per ITS 2013 guidelines, obtain MRI of the abdomen to assess for the presence of angiomyolipoma and renal cysts in patients with suspected or newly diagnosed tuberous sclerosis. E As per ERS 2010 guidelines, obtain abdominopelvic CT at diagnosis or during work-up to identify angiomyolipoma and other abdominal lesions in all patients with suspected or confirmed pulmonary lymphangioleiomyomatosis. B Show 3 more Renal function tests: assess GFR for the evaluation of renal function in patients with suspected or newly diagnosed tuberous sclerosis. E https://web.pathway.md/diseases/reczRRH4FO9lCvn5R 1/4 6/24/23, 2:57 PM Renal angiomyolipoma Pathway Screening for tuberous sclerosis: Screen for tuberous sclerosis, with further lymphangioleiomyomatosis screening if tuberous sclerosis complex is present, in patients with bilateral angiomyolipoma. B Refer patients with bilateral angiomyolipoma and lymphangioleiomyomatosis, and other patients where doubt remains, to a clinical geneticist for a full evaluation. B Screening for pulmonary lymphangioleiomyomatosis: consider obtaining HRCT screening for lymphangioleiomyomatosis in female patients with unilateral angiomyolipoma, no clinical features of tuberous sclerosis, and no pulmonary symptoms. C Screening for hypertension: obtain an accurate BP measurement to screen for hypertension in patients with suspected or newly diagnosed tuberous sclerosis involving kidneys. E 2. Medical management mTOR inhibitors: As per ITS 2013 guidelines, initiate mTOR inhibitors as first-line therapy in patients with asymptomatic, growing angiomyolipoma measuring > 3 cm in diameter. E As per ERS 2010 guidelines, do not use mTOR inhibitors as first-line therapy in patients with renal angiomyolipoma. Consider initiating sirolimus on an individual basis in patients with symptomatic angiomyolipoma or lymphangioleiomyomatosis-related masses not amenable to embolization or conservative surgery in experienced centers. D 3. Therapeutic procedures Embolization: As per ITS 2013 guidelines: Perform embolization followed by corticosteroids as first-line therapy in patients with angiomyolipoma presenting with acute hemorrhage. E Consider performing selective embolization as second-line therapy in patients with asymptomatic angiomyolipoma. E As per ERS 2010 guidelines, consider performing embolization in patients with asymptomatic renal angiomyolipomas > 4 cm or with renal aneurysms > 5 mm in diameter. E Show 2 more 4. Surgical interventions Nephron-sparing surgery: As per ITS 2013 guidelines: Avoid performing nephrectomy in patients with angiomyolipoma presenting with acute hemorrhage. D https://web.pathway.md/diseases/reczRRH4FO9lCvn5R 2/4 6/24/23, 2:57 PM Renal angiomyolipoma Pathway Consider performing nephron-sparing resection as second-line therapy in patients with asymptomatic angiomyolipoma. E As per ERS 2010 guidelines, consider performing nephron-sparing surgery in patients with asymptomatic renal angiomyolipomas > 4 cm or with renal aneurysms > 5 mm in diameter. E Show 2 more 5. Patient education General counseling: Advise patients to seek urgent medical attention in the presence of symptoms of bleeding angiomyolipoma. B Inform pregnant patients, ideally pre-pregnancy or as soon after conception as feasible, about the risk of bleeding from angiomyolipoma. E 6. Follow-up and surveillance Serial clinical and laboratory assessment: assess renal function (including determination of GFR) and BP at least annually in patients with possible or definite tuberous sclerosis involving kidneys. E Serial imaging assessment: As per ITS 2013 guidelines, obtain MRI of the abdomen to assess for the progression of angiomyolipoma and renal cystic disease every 1-3 years throughout the lifetime of patients with possible or definite tuberous sclerosis. E As per ERS 2010 guidelines: Do not treat patients with asymptomatic renal angiomyolipoma < 4 cm, rather obtain annual ultrasound assessments unless symptoms occur. Obtain CT or MRI if ultrasound measurements are unreliable due to technical factors. D Obtain ultrasound assessment twice yearly in patients with asymptomatic renal angiomyolipomas > 4 cm or with renal aneurysms > 5 mm in diameter, to evaluate growth as they are at increased risk of bleeding. E References 1. Darcy A Krueger, Hope Northrup, International Tuberous Sclerosis Complex Consensus Group. Tuberous sclerosis complex surveillance and management: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol. 2013 Oct;49 4 255 65. Open 2. S R Johnson, J F Cordier, R Lazor et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010 Jan;35 1 14 26. Open 3. B. Ljungberg Chair), L. Albiges, J. Bedke et al. EAU Guidelines on Renal Cell Carcinoma. EAU. 2022. Open https://web.pathway.md/diseases/reczRRH4FO9lCvn5R 3/4 6/24/23, 2:57 PM Renal angiomyolipoma Pathway 4. Antonio Finelli, Nofisat Ismaila, Bill Bro et al. Management of Small Renal Masses: American Society of Clinical Oncology Clinical Practice Guideline. J Clin Oncol. 2017 Feb 20;35 6 668 680. Open https://web.pathway.md/diseases/reczRRH4FO9lCvn5R 4/4