Rare Presentation of Intravenous Leiomyomas with Intracardiac Leiomyomatosis

Uterine leiomyomas, or fibroids, are the most prevalent benign tumor pathology affecting women.1 Fibroids are reportedly found in nearly 70% of Caucasian women and more than 80% of women with African ancestry.1 Rarely, fibroids progress to uterine intravenous leiomyomas (IVL), a neoplastic growth pattern characterized by intravascular proliferation of this smooth muscle cell tumor.2 This tumor is typically confined to the pelvic vasculature but can occasionally spread with venous blood flowing to the heart, ultimately penetrating the inferior vena cava, right heart chambers, and pulmonary arteries.3 If the tumor reaches the heart, this intracardiac leiomyomatosis (ICL) can result in severe cardiac symptoms including arrythmias, murmurs, and sudden death.3,4 The case presentation shows both IVL and ICL in a patient. Approximately 12 months following hysterectomy due to fibroids, the patient presented with mild dyspnea on exertion, retrosternal chest pain, and a syncopal episode. She was diagnosed with saddle embolism with extension into right ventricle and complete occlusion of the inferior vena cava (IVC). Informed by imaging from echocardiogram, computed tomography (CT) angiogram of the chest, CT of the abdomen and pelvis with contrast and magnetic resonance imaging (MRI) of the abdomen and pelvis, providers proceeded with mechanical thrombectomy followed by IVC filter, tumor debulking surgery, resection of a pelvic mass, and proximal vaginectomy. To facilitate early detection and treatment, providers should factor the risk of IVL and ICL into ongoing care and patient education post hysterectomy or fibroid diagnosis.