Renal angiomyolipoma (RAML), though a rare benign tumor, may impose a significant morbidity or even mortality due to its unique characteristics and the complications subsequent to its treatment. The classic tumor variant is composed of smooth muscular, vascular, and fatty components. The most straightforward diagnosis is when the fat component is abundant and gives a characteristic appearance on different imaging studies. In fat-poor lesions, however, the diagnosis is difficult and presumed a renal cell carcinoma. Yet, some variants of RAML, though rare, express an aggressive behavior leading to metastasis and mortality. The challenge lies in the early detection of benign variants and identifying aggressive lesions for proper management. Another challenge is when the vascular tissue component predominates and poses a risk of hemorrhage that may extend to the retroperitoneum in a massive life-threatening condition. The predicament here is to identify the characteristics of tumors at risk of bleeding and provide a prophylactic treatment. According to the clinical presentation, different treatment modalities, prophylactic or therapeutic, are available that span the spectrum of observation, embolization, or surgery. Renal impairment may result from extensive tumor burden or as a complication of the management itself. Improvement of diagnostic techniques, super-selective embolization, nephron-sparing surgery, and late treatment with the mammalian target of rapamycin inhibitors have provided more effective and safe management strategies. In this review, we examine the evidence pertaining to the risks imposed by RAML to the patients and identify merits and hazards associated with different treatment modalities.
Keywords: angiomyolipoma; embolization; hemorrhage; kidney; nephrectomy.