Abstract
Intravenous leiomyomatosis (IVL) is a benign smooth muscle tumor which may cause fatal outcome. Furthermore, the clinical manifestations are variable and nonspecific, typically resulting in misdiagnosis. Computerized Tomography (CT) played a vital role in the diagnostic process and pre-surgical assessment. Here we describe one case of IVL extending from the inferior vena cava to the right atrium and ventricle, then review the literature and discuss the imaging findings and clinical manifestation of this tumor. It is suggestive for clinicians to be aware of this condition and highly suspect a middle-aged woman with a history of uterine leiomyoma presenting with an inferior vena cava filling defect or mass shadow to early diagnose and treat.
Highlights
Intravenous leiomyomatosis (IVL) is a rare histologically benign tumor originated in the uterine mesoderm, but it is behaviorally malignant, potentially life threatening tumor with unpredictable biological behavior, due to growing and extension with the intrauterine venous system
Though the organizational structure of the tumor is the hyperplasia of smooth muscle cell, which is similar to leiomyoma of uterus, it is completely different from uterine leiomyoma considering it’s malignant growth way [3]. 10%~30% of affected patients has complication in postcava, right atrium and lung, and it may cause death in some severe circumstance [4]
Etiology and Pathogenesis IVL is a rare benign tumor that is histologically characterized by nodular masses of smooth muscle cells growing within systemic veins
Summary
Intravenous leiomyomatosis (IVL) is a rare histologically benign tumor originated in the uterine mesoderm, but it is behaviorally malignant, potentially life threatening tumor with unpredictable biological behavior, due to growing and extension with the intrauterine venous system. Smooth muscle cell spreading along blood vessel like nodule is the main manifestation. Though the organizational structure of the tumor is the hyperplasia of smooth muscle cell, which is similar to leiomyoma of uterus, it is completely different from uterine leiomyoma considering it’s malignant growth way [3]. Due to its rarity and the non-specific clinical manifestations, IVL is frequently misdiagnosed as malignant thrombus or thrombosis. To explore the related clinical manifestation and useful examination is the focus of clinicians. We report a case of IVL with large vascular involvement and the appearance of Computerized Tomography (CT) in the chest, through which clinicians can get some useful information
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