An unusual appearance of renal epithelioid angiomyolipoma

Epithelioid angiomyolipoma is a recently described rare variant of renal angiomyolipoma. It can occur in patients with or without tuberous sclerosis. We report the imaging findings of a case of epithelioid angiomyolipoma that showed the presence of fatty tissue undifferentiated from the typical angiomyolipoma at the beginning. After partial nephrectomy, tumour recurrence occurred two years later, presenting as completely solid tumours with no adipose tissue, and with invasion into the psoas muscle and left adrenal gland. Differentiation of this tumour from renal cell carcinoma is difficult. Both the radiologist and surgeon should be aware of the existence of this tumour and its potentially malignant behaviour.