Renal epithelioid angiomyolipoma (E-AML) is a rare variant of angiomyolipoma (AML).
It is a mesenchymal tumour believed to originate from the perivascular epithelioid cell (PEC). Unlike conventional AML which are benign, E-AML has a rare aggressive behaviour. Conventional AML is typically triphasic containing adipose tissue, smooth muscle and dystrophic vessels in variable proportions, while E-AML are generally composed of plump spindled and polygonal-shaped "epithelioid cells" showing clear or eosinophilic cytoplasm and occasional pleomorphic multinucleated giant cells. E-AML can be misdiagnosed as renal cell carcinoma (RCC) when these "epithelioid cells" show clearing. Only a small number of cases of E-AML have been reported with the standard treatment being radical or partial nephrectomy. We report the first case report of a surgeon-controlled robotic partial nephrectomy using a near-infrared fluorescence imaging using indocyanine green dye on a 25-year-old woman with a T1B (6.6 cm) right renal mass. The final pathology revealed the diagnosis of E-AML. There was no recurrence and metastases after the 6-month follow-up.
Written by:
Patel TH, Sirintrapun SJ, Hemal AK. Are you the author?
Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, North Carolina, USA.
Reference: Can Urol Assoc J. 2012 Apr;6(2):E91-4.
doi: 10.5489/cuaj.11250
PubMed Abstract
PMID: 22511444
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