Intravascular leiomyoma with intracardiac extension associated with hepatorenal polycystic disease

We present the case of a woman with hepato-renal polycystic disease and chronic hemodialysis admitted for paroxysmal atrial fibrillation. A right heart mass was diagnosed by echocardiography. MRI and angio-CT scans confirmed a calcified tumor with the origin in the right internal iliac vein that extended through inferior vena cava to the right cardiac chambers. The intracardiac tumor was excised by cardiovascular surgery due to perceived pulmonary embolic risk. The tumor mass was a leyomyoma. Despite extensive inferior vena cava extension, tumor dimensions did not increase at one year follow-up and no intracardiac recurrence was noticed.