A 41-year-old woman was admitted to our hospital with a 3-week history of swollen legs and abdominal distension that persisted after diuretic therapy. Physical examination revealed a blood pressure of 110/70 mm Hg, a heart rate of 76 bpm, and a respiratory rate of 15 breaths per minute. Cardiac auscultation was remarkable for a tricuspid pansystolic regurgitation murmur. On abdominal examination, a nontender mass resembling a uterus 12 to 13 weeks pregnant was noted in the lower abdomen. An ECG showed sinus rhythm with nonspecific findings, and the chest radiograph was normal. An abdominal ultrasound revealed an elongated inferior vena cava (IVC) and a filling defect inside the vein suggesting thrombosis. A CT scan revealed a large, heterogeneous, and irregular pelvic mass arising from the uterus (Figure 1) and a thrombus-like image extending from the IVC into the left renal vein (Figure 2) and up the right atrium. Echocardiography showed a mobile mass extending from the IVC through the right atrium and right ventricle (Figure 3), with its apparent tip moving within the pulmonary valve, producing tricuspid regurgitation.A presumptive diagnosis of uterine intravenous leiomyomatosis was made. MRI demonstrated a large mass in the uterus extending via the left iliac vein and the inferior vena cava into the right cardiac cavities, with the same signal intensity through the full extent of the tumor (Figure 4). Angiography of the inferior vena cava and iliac veins revealed almost complete occlusion of the IVC, with prominent collateral circulation ( Figure 5). Tumors in the heart and inferior vena cava were successfully removed under deep hypothermia and circulatory arrest (Figures 6 and 7).

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