A massive renal epithelioid angiomyolipoma with multiple metastatic lymph nodes

A 47-year-old man presented with dull pain in the left upper abdomen for 1 year. Computed tomograph (CT) examination revealed a very large heterogeneously enhancing mass in the left kidney, measuring up to 28 cm. The mass was accompanied by several enlarged lymph nodes in the peri-aortic region. Radical nephrectomy was performed and pathologic evaluation revealed sheets of epithelioid cells and piecemeal necrosis consistent with malignant epithelioid angiomyolipoma (EAML) with regional lymph node metastases. The tumor cells were strongly positive for human melanosome-associated protein (HMB-45) on immunohistochemical staining. There was neither metastasis nor recurrence 2 years postoperatively. EAML is a rare tumor of mesenchymal tissue with potential for aggressive behavior. If this neoplasm is suspected based on CT features, EAML should be confirmed by pathology and immunohistochemistry.