Angiomyolipoma is a member of a family of tumors called perivascular epithelioid cell tumors. Renal angiomyolipoma (AML) should be occured with tuberous sclerosis complex or pulmonary lymphangioleiomyomatosis or occured as a sporadic AML. Renal AMLs are found in 0.3% to 2.1% of kidneys at autopsy. About 80% of AMLs are sporadic and not associated with any genetic syndrome. There are two histologic types of AMLs; classic and epithelioid. Classic AMLs are benign but epithelioid type occasionally undergoes malignant transformation. The main risk of renal AML is the rupture with massive retroperitoneal bleeding, called Wunderlich syndrome which may occur in pregnants frequently. The diagnosis of renal AML is generally made by imaging studies (usually ultrasound, computed tomography or magnetic resonance imaging (MRI)). If it is not possible to diagnose renal AML with imaging studies, percutaneous needle biopsy may be required. Potential interventions include nephron sparing surgery, selective renal artery embolisation, radiofrequency ablation and total nephrectomy. Prophylactic surgery is generally recommended in patients with AMLs larger than 4 cm diameter or those with high vascularity and/or an aneurysm measured ≥5 mm to prevent spontaneous hemorrhage.

Keywords: Renal angiomyolipoma, tuberosclerosis, retroperitoneal hemorrhage