2022-RA-1408-ESGO Intracardiac intravenous leiomyomatosis with malignant hystological features

Introduction/Background Intravenous leiomyomatosis (IVL) is defined by the presence of smooth muscle nodules beyond vessels an uterine leiomyoma and into extrauterine venous system. Its extension right-sided cardiac chambers can be fatal. Most cases lack marked nuclear atypia or elevated mitotic activity. Transition from a non-invasive to invasive IVL not clearly understood. The objective report patient with intracardiac malignant histological features consistent sarcoma. Methodology 63 year old female, hemodynamically stable, referring painful mass that occupied pelvic cavity up hypogastrium, associated abnormal bleeding. Abdominal-pelvic magnetic resonance image (MRI) reported solid tumor originating in posterior wall uterus, extended through right external iliac vein atrium (RA) partially occupying inferior vena cava (IVC) common vein. Chest computed tomography showed no pulmonary metastasis. Cardiac MRI intravascular thrombus tumoral origin IVF RA protruding tricuspid valve (TV) ventricule (RV). Tran-sesophageal echocardiography revealed 37x13 mm vascularized irregular extending IVC going TV RV, effect on valvular function. Results A one stage procedure was performed including resection, hysterectomy, adnexectomy, dissection removal endovascular tumor. Due nonsurgical bleeding, mediastinal packing necessary unpacking 24 hours later. Final pathology examination high grade sarcomatous within findings IVL. Conclusion has quasi-malignant behavior. Complete impacts prognosis recurrence rates. Reports are scarce.