Intravascular Leiomyomatosis with Intracardiac Extension

Intravenous leiomyomatosis (IVL) is a rare nonmalignant tumor defined as a benign smooth muscle cell neoplasia in the veins. Patients with IVL may present with symptoms of a uterine leiomyoma such as pelvic pain and vaginal bleeding, or cardiorespiratory symptoms, including dyspnea and leg swelling. We report the case of a 65-year-old otherwise healthy Caucasian woman. Past medical history consisted of hysterectomy and left salpingo-oophorectomy 15 years before for multiple uterine fibromyomas associated with leiomyoma of vascular origin. A thoracoabdominal computed tomography (CT) scan confirmed the presence of a mass, measuring 76 × 37 × 44 mm, arising from the inferior vena cava (IVC) at the level of the left renal vein extending all the way into the right atrium and right ventricle. At laparotomy, a tumoral mass was excised from the left broad ligament up to the left renal vein and from the IVC up to its retrohepatic tract. Sternotomy was performed and cardiopulmonary bypass (CPB) was established among ascending aorta, upper vena cava, and right common femoral vein. After atriotomy, a voluminous and firm mass was excised from the right atrium, down to the level of the IVC. CPB was maintained for 80 min. Perioperative transfusion included two plasma and two red blood cells units. No adjuvant treatment was administered. Follow-up with annual CT scans was performed. Patient had no signs of recurrence after 3 years.