Management of Intravenous Leiomyomatosis: A Comprehensive Review of Surgical and Perioperative Considerations

Intravenous leiomyomatosis is a rare condition in which a smooth muscle tumor originates from the uterus and extends into the pelvic and systemic vasculature, frequently involving the inferior vena cava and the right atrium. Despite its benign histology, intravenous leiomyomatosis poses significant clinical challenges due to its potential to cause life-threatening complications. Conventional management involves a combined surgical approach: intracardiac tumor resection performed via sternotomy, with abdominal and pelvic tumor removal, including hysterectomy, conducted through laparotomy. Alternatively, an abdominal-only approach allows for complete tumor resection through inferior vena cava incision and hysterectomy without sternotomy. Surgical timing is an important consideration, with single-stage procedures addressing all tumor components in 1 operation, while 2-stage procedures separate cardiac and abdominal/pelvic resections into distinct surgeries to reduce risks in patients with extensive cardiac involvement or limited surgical tolerance. Post-operative management includes careful resumption of anti-coagulation for several months to prevent thromboembolic complications, particularly in patients with vascular involvement. Hormonal therapy, such as aromatase inhibitors, is considered for patients with residual disease. Advanced imaging techniques, including magnetic resonance imaging, computed tomography, and echocardiography, are essential in both preoperative planning and post-operative surveillance to ensure optimal surgical strategy and to help monitor for residual tumors. Multidisciplinary collaboration is crucial in the management of intravenous leiomyomatosis, ensuring a comprehensive approach that optimizes patient outcomes.