The 2004 World Health Organization Classification of Renal Neoplasms defined epithelioid angiomyolipoma as a potentially malignant mesenchymal neoplasm, characterized by a proliferation of predominantly epithelioid cells with approximately one third of patients experiencing metastases and one half of them having a history of tuberous sclerosis complex. Observations: We report two cases of renal epithelioid angiomyolipoma diagnosed at our institution in order to analyze their clinical behaviour

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... nd histopathological features, and insist on diagnostic pitfalls. Conclusion: Renal tumours with certain unusual features should be investigated immunohistochemically to exclude the possibility of epithelioid angiomyolipoma. These tumours are more likely to have an aggressive behaviour when they show more morphologic features predicting malignancy.