Fig. 2: Structured approach to risk assessment and treatment of angiomyolipoma haemorrhage.

In cases of acute haemorrhage of an angiomyolipoma, radiological intervention should be considered as the first-line approach. If not directly available, patient management should not be delayed and surgery should be initiated (employing a nephron-sparing approach, if possible, in the acute setting). Upon determination of substantial bleeding risk of an angiomyolipoma (Box 5), mechanistic target of rapamycin complex 1 (mTORC1) inhibition is the first-line approach. If no response to this treatment is observed or its further use is limited by side effects, reassessment and consideration of alternative strategies are required. TSC, tuberous sclerosis complex. ^aWith haemodynamic compromise or without spontaneous termination; in cases of moderate self-limiting bleeding, medical (intensive care or transfusion) or conservative management might be an option.