Intravenous Leiomyomatosis with Intracardiac Extension: Echocardiographic Study and Literature Review

Abstract

I ntravenous leiomyoma (IVL) is a rare, histologically benign smooth-muscle-cell tumor that occurs only in women. This neoplasm occupies vascular spaces from the intrauterine venules to the systemic veins, including the iliac vein and inferior vena cava (IVC), and it does not invade the tissue. The mass can extend into the right heart chambers and pulmonary arteries. 1,2 Its extrauterine involvement occurs in approximately 30% of cases, and intracardiac extension accounts for about 10%. [3][4][5] This extension of IVL into the right side of the heart is called intracardiac leiomyomatosis (ICL).The diagnosis of ICL can be overlooked. Echocardiography, abdominal ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) are available for detection and diagnosis. Echocardiography is important in the initial diagnosis of ICL. To our knowledge, the literature about ICL chiefly comprises case reports, and the authors of the few case series have not in general discussed the echocardiographic characteristics and extending pathways of ICL. We retrospectively studied the cases of 7 patients with ICL who underwent successful tumor resection in our hospital. We outlined the echocardiographic characteristics of the tumors and analyzed their clinical features, confirmed the extending pathways by means of CT reports, and studied the surgical and pathologic results. We discuss the echocardiographic diagnosis of ICL and briefly review the pertinent medical literature. Patients and MethodsWe reviewed our hospital's clinical database and identified 7 women who had undergone surgical resection of ICL tumors from January 2003 through July 2012. The echocardiographic images included parasternal, apical, and subcostal views. In addition, M-mode, pulsed and continuous-wave Doppler, and color-flow Doppler images

Cardiovascular Ultrasonographic Features and Clinical Findings for Intravenous Leiomyomatosis: A Case Report

“…IVL can follow 2 distinct pathways into the systemic venous circulation (11): extension via the uterine vein or the ovarian vein. The uterine vein is the principal pathway and can extend through this vessel into the internal iliac veins, the common iliac veins, and finally into the IVC.…”

Post-hysterectomy intravenous leiomyomatosis: A case of successful multidisciplinary surgery under non-extracorporeal circulation

“…When leiomyomatosis affects the spine, magnetic resonance imaging may provide information for the diagnosis and the extent of the lesions (8). Echocardiography with good penetration of the tumor is also helpful in reaching a diagnosis (9). …”

Multiservice single-stage surgery for intracardiac leiomyomatosis

“…Computed tomograms also reveal pelvic masses extending into the right internal and common iliac veins with continuation into the inferior vena cava. [13] Surgery can be performed via a single or two-stage operation. The two-stage procedure involves first performing the sternotomy to remove the intracardiac mass to relieve the patient of symptoms and decrease the risk of heart failure and cardiac arrest.…”