Epithelioid Angiomyolipoma ? The Treatment Conundrum

A 21 year old female was transferred to our institution with generalised abdominal pain and an incidental finding of an exophytic 4cm enhancing upper pole renal mass on Computerised Tomography (CT). Her abdominal pain was subequently diagnosed as being secondary to constipation. The diagnosis of a likely Renal Cell Carcinoma (RCC) was explained to the patient and she was booked for a laprascopic mobilisation open partial nephrectomy. Histologic examination demonstrated adipocytes, spindle cells and abnormal vessels. The tumour tested postive for melanocytic and smooth muscle markers. Ten percent of the tumour consisted of epithelioid cells with scattered mitotic figures. The diagnosis was that of a fat poor angiomyolipoma (AML) with a small focus of epithelioid AML. Resection was clear of margins. We elected to surveil rather than electively add adjuvant therapies. At 12 month follow-up the patient remains disease free.