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Renal angiomyolipomas are benign mesenchymal tumours. Whether isolated or associated with phacomatosis, they are histologically identical, but it is often difficult to distinguish an isolated angiomyolipoma from renal cancer. When it is combined with phacomatosis, it is frequently bilateral and can provoke retroperitoneal haemorrhages. These peculiarities qualify the therapeutical management which would a priori be conservative in the case of a benign tumour.
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