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Angiomyolipoma of the kidney is a neoplasm composed of variable amounts of mature adipose tissue, smooth muscle, and thick-walled blood vessels derived from perivascular epithelioid cells (1â3). Its prevalence in the general hospital population has been reported to be 0.13% in Japan (4).
The article reviewed. A Rare Case of Metastatic Renal Epithelioid Angiomyolipoma. The authors present an interesting case of a very rare renal neoplasm, malignant epithelioid angiomyolipoma (AML), which belongs to a family of mesenchymal tumors known as perivascular epithelioid tumors (PEComas).