Intravascular Leiomyomatosis Extending to Right Atrium: A Rare Caused Syncope

Intravascular leiomyomatosis (IVL) is a variant of leiomyoma characterized by intravascular proliferation of a histologically benign smooth muscle tumor extending beyond the uterus into distant great vessels or the heart. It is a rare disease and results in death. Here, we reported the case of 48-year-old, otherwise well woman, who presented to the emergency department with syncope. Pulmonary computed tomography (CT) demonstrated a large low-density shadow originating from the inferior vena cava (IVC) extending into the right atrium (RA). Magnetic resonance venography (MRV) showed that a neoplasm was "snakelike," which completely occluded the right internal iliac vein (RIIV), the common iliac vein (CIV), and IVC. A multidisciplinary team of specialists consisting of vascular surgeons, cardiac surgeons, gynecologists, anesthesiologists, and radiologists reviewed the history, clinical examination findings, and diagnostic imaging of the patient. A decision was made to proceed with one-stage surgery (resection of thoracoabdominal tumor extension at one operative setting). After surgery, the patient's vital signs were restored, and her symptoms were disappeared. She was discharged on hospital day 21 without complications. One-stage surgical approach to completely remove an IVL with RA involvement is an optimal choice if the patient's physical condition permits. Intravascular leiomyomatosis (IVL) is a variant of leiomyoma characterized by intravascular proliferation of a histologically benign smooth muscle tumor extending beyond the uterus into distant great vessels or the heart. It is a rare disease and results in death. Here, we reported the case of 48-year-old, otherwise well woman, who presented to the emergency department with syncope. Pulmonary computed tomography (CT) demonstrated a large low-density shadow originating from the inferior vena cava (IVC) extending into the right atrium (RA). Magnetic resonance venography (MRV) showed that a neoplasm was "snakelike," which completely occluded the right internal iliac vein (RIIV), the common iliac vein (CIV), and IVC. A multidisciplinary team of specialists consisting of vascular surgeons, cardiac surgeons, gynecologists, anesthesiologists, and radiologists reviewed the history, clinical examination findings, and diagnostic imaging of the patient. A decision was made to proceed with one-stage surgery (resection of thoracoabdominal tumor extension at one operative setting). After surgery, the patient's vital signs were restored, and her symptoms were disappeared. She was discharged on hospital day 21 without complications. One-stage surgical approach to completely remove an IVL with RA involvement is an optimal choice if the patient's physical condition permits.