Angiomyolipoma is a mesenchymal tumor composed of varying proportions of dysmorphic blood vessels, adipose tissue and smooth muscle. Angiomyolipoma is a member of the PEComa (perivascular epithe-lioid cell tumor) family, which additionally includes lymphangiomyomatosis, clear cell "sugar" tumor of the lung, and morphologically and immunohisto-chemically related tumors at other sites. In about 8% of cases an epithelioid component comprises more than 10% of the tumor and such tumors are often designated as epithelioid angiomyolipoma. Only about 1% of renal angiomyolipomas show purely epithelioid morphology.
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