An unusual cause of hepatic venous outflow obstruction: Epithelioid angiomyolipoma of the kidney

Abstract

Epithelioid angiomyolipoma (EAML) is a rare, aggressive perivascular epithelioid cell tumour (PEComa) with malignant potential. A 36-year-old man presented with a large hypervascular left renal mass. Computed tomography showed a 12.5 cm heterogeneous tumour with thrombus extending from the renal vein into the inferior vena cava and right atrium, complicated by Budd–Chiari syndrome. Biopsy confirmed Melan-A–positive epithelioid cells. Epithelioid angiomyolipoma can mimic advanced renal cell carcinoma. Management included selective transarterial angioembolisation to reduce haemorrhage risk. Contribution: This report documents a rare EAML presentation complicated by Budd–Chiari syndrome, highlighting the utility of pre-procedural angioembolisation for managing aggressive, hypervascular variants with extensive venous involvement.