Resection of a staggering 36-cm angiomyolipoma

Angiomyolipomas (AMLs) are benign tumours characterized by fat, smooth muscle and vascular components. Epithelioid AML is a recognized variant of AML that is comprised of epithelioid smooth muscle cells. We present a case of a 41-year-old male who presented with light-headedness, dizziness, right-sided abdominal pain and, on subsequent computed tomography, was found to have an enormous right kidney mass characteristic of an AML. The patient underwent preoperative selective arterial embolization followed by a right radical nephrectomy. The pathology revealed a 36-cm AML with focal epithelioid features. Although uncommon, AMLs can present as enormous retroperitoneal masses.