Intravenous leiomyomatosis (IVL) is a relatively novel disease and can be aggressive. Since the first case was reported, it has a history of more than one hundred years, but the clinical incidence is extremely low, which has profound clinical research significance. Early recognition and management can prevent fatal consequences. IVL should be considered in female patients with a history of leiomyomas and intravascular filling defects. Because the patient's symptoms are not obvious, it is often misdiagnosed. We report on a female patient with IVL who presented with dizziness, and discuss the unexplained cause of filling defects within the right cardiac chambers and pulmonary arterial system. We present a complicated and rare case of IVL in a woman with a right atrial mass. A long, cord-like mass with inhomogeneous echo was detected by echocardiography. Coronary computed tomography (CT) showed a large hypodense mass occupying most of the right atrial cavity, spreading in both pulmonary arteries. Vascular ultrasound revealed a mixed echogenic mass invading the inferior vena cava like a rope. We performed surgery on her and complete resection on the tumor. We search for documents by searching databases such as National Center for Biotechnology Information (NCBI), China National Knowledge Infrastructure (CNKI) and Web of Science (WoS), etc. the recurrence rate recorded in the 4 cases series was 47.83% (11/23), 43.48% (20/46), 12.50% (2/16), and 10.00% (2/20), respectively.
Keywords: Intravenous leiomyomatosis (IVL); case report; literature review; right atrial mass; surgery.