[Renal angiomyolipoma causing inferior vena cava thrombus and secondary Budd-Chiari's syndrome].

OBJECTIVE

We report one case of renal angiomyolipoma with three characteristics: renal sinus origin, inferior vena cava tumor thrombus reaching the right atrium and Budd-Chiari syndrome.

METHODS

The characteristics of the case are presented and discussed.

RESULTS/CONCLUSIONS: Renal angiomyolipoma is a benign mesenchymal tumor. It is an uncommon tumor (2-6.4% of all kidney tumors), with a female predominance. Angiomyolipoma most often originate from the renal parenchyma but they can rarely originate from the renal sinus. They can involve the renal vein, the inferior vena cava and even the right atrium as a tumor thrombus. Angiomyolipoma commonly present as an incidental finding on radiographic studies, but the clinical presentation varies from flank pain to fatal pulmonary tumor embolism. The Budd-Chiari syndrome is an extremely rare presentation. Because of the risk of potentially fatal cardiopulmonary embolism and death, surgical treatment (radical nephrectomy plus tumor thrombectomy) of these lesions is indicated even when they are asymptomatic.