Abstract
An adult female with abdominal discomfort was found to have a renal mass and paraortic lymphadenopathy on computed tomography of the abdomen and pelvis. The tumor was resected and diagnosed as malignant epithelioid angiomyolipoma (malignant perivascular epithelioid cell neoplasm) of the kidney. The tumor was associated with matted lymph node metastasis.
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