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Renal angiomyolipoma

Guidelines

Key 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). ...
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Diagnostic investigations

Diagnostic imaging: as per TSCi 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.
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  • Renal function tests

  • Screening for tuberous sclerosis

  • Screening for pulmonary lymphangioleiomyomatosis

  • Screening for hypertension

Medical management

mTOR inhibitors: as per TSCi 2013 guidelines, initiate mTOR inhibitors as first-line therapy in patients with asymptomatic, growing angiomyolipoma measuring > 3 cm in diameter.
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Therapeutic procedures

Embolization
As per TSCi 2013 guidelines:
Perform embolization followed by corticosteroids as first-line therapy in patients with angiomyolipoma presenting with acute hemorrhage.
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Consider performing selective embolization as second-line therapy in patients with asymptomatic angiomyolipoma.
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Surgical interventions

Nephron-sparing surgery
As per TSCi 2013 guidelines:
Avoid performing nephrectomy in patients with angiomyolipoma presenting with acute hemorrhage.
D
Consider performing nephron-sparing resection as second-line therapy in patients with asymptomatic angiomyolipoma.
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Patient education

General counseling
As per ERS 2010 guidelines:
Advise patients to seek urgent medical attention in the presence of symptoms of bleeding angiomyolipoma.
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Inform pregnant patients, ideally pre-pregnancy or as soon after conception as feasible, about the risk of bleeding from angiomyolipoma.
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Follow-up and surveillance

Serial clinical and laboratory assessment: as per TSCi 2013 guidelines, assess renal function (including determination of GFR) and BP at least annually in patients with possible or definite tuberous sclerosis involving kidneys.
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More topics in this section

  • Serial imaging assessment