Intravascular Leiomyomatosis with Intracardiac Extension

International audienceIntravenous leiomyomatosis (IVL) is a rare non-malignant 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 cardio-respiratory symptoms, including dyspnoea and legs swelling. We report the case of a 65 years-old otherwise healthy Caucasian woman. Past medical history consisted of hysterectomy and left salpingo-oophorectomy 15 years before for multiples uterine fibromyomas associated with leiomyoma of vascular origin. A thoraco-abdominal CT scan confirmed the presence of a mass, measuring 76x37x44 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 tumoural mass was excised from the left broad ligament up to the left renal vein and from the IVC up to its retro-hepatic tract. Sternotomy was performed and cardiopulmonary bypass (CPB) was established between 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 minutes. 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 year