Intravenous leiomyomatosis (IVL) is a rare, benign tumor that originates from the uterus. IVL is usually confined to the pelvic venous system, but it travels into the inferior vena cava (IVC) in 10% of cases and even into the heart in 3% of cases. We present a case of successful resection of recurrent IVL with right atrium extension. We used only an abdominal incision and transesophageal echocardiography (TEE) guidance. A 40-year-old female patient visited our hospital with recurrent IVL. She had a history of total abdominal hysterectomy and right salpingo-oophorectomy due to IVL performed one year prior. On computed tomography (CT) and ultrasonography, IVL was found to involve both ovarian veins, the left renal vein, and the IVC extending to the right atrial junction. Using intraoperative TEE monitoring, we could see that IVL was not attached to the vascular wall. After creating a midline abdominal incision, we removed the tumor through the enlarged ovarian vein and ovary. The patient had an uneventful recovery and was discharged home on the ninth postoperative day.
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