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A case report: malignant epithelioid angiomyolipoma

A 55-year-old lady presented with a history of left sided abdominal pain, fever and recent 10 kg weight loss. Radiological study showed a left renal mass. Macroscopic examination revealed a 1284 g kidney with a large solitary lower pole tumour, measuring 170 mm in maximum dimension. The tumour demonstrated a variegated solid yellow cut-surface with extensive haemorrhage and necrosis. Light microscopy showed an oncocytic epithelioid tumour. The cells were large with abundant eosinophilic cytoplasm and pleomorphic nuclei with prominent nucleoli. In addition, there were scattered rhabdoid-like and ganglion-like cells with numerous admixed florette-type multinucleated giant cells. Mitotic figures were sparse. There were no areas of conventional angiomyolipoma. Immunohistochemically, the tumour was positive for Melan-A, HMB-45, E-cadherin, S100 and vimentin. They were negative for CK7, CK AE1/AE3, CD117, CD10, SMA and desmin. The morphological and immunohistochemical features were that of a malignant epithelioid angiomyolipoma. Epithelioid angiomyolipoma is a rare mesenchymal neoplasm with a malignant potential. It belongs to the PEComa family and mimics a variety of oncocytic or clear cell neoplasms. It often poses diagnostic difficulties and is thus an important entity to recognise in order to optimise patient management.