LAUSR

Angiomyolipoma (AML) is a type of tumor in the perivascular epithelioid cell neoplasm (PEComa) family. Epithelioid angiomyolipoma (EAML), having malignant potential, is considered a rare variant of angiomyolipoma. The most common site of EAMLs is kidney, and extra-renal EAMLs are very uncommon, with liver being the most common site.1 Other locations including retroperitoneum, uterus, liver, lung, breast, cardiac septum, pancreas, prostate, and gastrointestinal tract have also been described.2 To our acknowledgment, only 6 cases of retroperitoneal EAML was reported in the English literature.3 Presented here is a 46-year-old female with a giant (20 cm 15 cm x 15cm) retroperitoneal epithelioid angiomyolipoma.