Images in Cardiovascular Medicine Renal Angiomyolipoma With Cardiac Extension in Patient With Tuberous Sclerosis Complex

26-year-old woman with a clinical diagnosis of tuber-ous sclerosis complex, presenting the classical triad ofseizures, mental retardation, and facial angiofibromas andreporting tachycardia, dyspnea during moderate physicalactivity, and 3 episodes of syncope in the past 30 days, wasadmitted to our hospital.Echocardiography revealed right cardiac chambers withincreased dimensions related to a large echogenic tumor withan inferior component within the inferior vena cava (Figure1). Computed tomography of the chest and abdomen withoutiodinated venous contrast media depicted a large tumorarising from the right kidney with adipose tissue, extendingsuperiorly via inferior vena cava with right cardiac chambersinvolvement (Figure 2). MRI of the abdomen and heartshowed the same large right kidney tumor with high signalintensity in T1- and T2-weighted images, signal intensity lossin T2-weighted images with fat saturation, and enhancementafter the use of venous paramagnetic contrast media (Figure3 and Movies I, II, and III in the online-only DataSupplement).The patient underwent surgical treatment with right ne-phrectomy and tumor resection. Pathology showed a angio-myolipoma with a cylindrical shape measuring 16 4 4 cm,and there were no atypical or epithelioid features seen onhistological examination (Figure 4).Renal angiomyolipoma (AML) is a benign neoplasm of thegroup of perivascular epithelioid cell tumors, consisting of 3elements:smoothmuscle,adiposetissue,andbloodvessels.Itis predominantly seen in women, and its incidence is highestin the fifth and sixth decades of life. It is well associated withtuberous sclerosis, seen in 80% of the patients. Typically,AML is confined to the kidney, but, in rare circumstances, thetumor can extend beyond the kidney.