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Epithelioid angiomyolipoma (EAML) of the kidney, in contrast to classic benign renal angiomyolipoma, is a rare mesenchymal neoplasm with malignant potential. Represent-ing a member of the perivascular epithelioid cells (PEComa) tumor family aris-ing from the perivascular epithelioid cells, its accurate dia-gnosis and therapeutic approach remains challenging.

Diagnostic Challenges and Extraordinary Treatment Response in Rare Malignant PEComa Tumor of the Kidney