Volfson Emily, Moshkovich Michal, Koen Johannes, Cusimano Robert, Kim Rachel Soyoun
University of Toronto, Temerty Faculty of Medicine, Toronto, ON, Canada.
University of Toronto, Toronto General Hospital, Peter Munk Cardiac Centre, Division of Cardiovascular Surgery, Toronto, ON, Canada.
Int J Gynecol Cancer. 2025 Jul 10;35(10):102009. doi: 10.1016/j.ijgc.2025.102009.
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.
静脉内平滑肌瘤病是一种罕见疾病，其中平滑肌肿瘤起源于子宫并延伸至盆腔和全身血管系统，常累及下腔静脉和右心房。尽管其组织学表现为良性，但静脉内平滑肌瘤病因其可能导致危及生命的并发症而带来重大临床挑战。传统治疗方法采用联合手术方式：通过胸骨切开术进行心内肿瘤切除，同时通过剖腹术进行腹部和盆腔肿瘤切除，包括子宫切除术。或者，单纯腹部手术方法可通过下腔静脉切口和子宫切除术在不进行胸骨切开术的情况下完成肿瘤完全切除。手术时机是一个重要考虑因素，一期手术在一次手术中处理所有肿瘤成分，而二期手术将心脏和腹部/盆腔切除分为不同手术，以降低心脏受累广泛或手术耐受性有限患者的风险。术后管理包括数月内谨慎恢复抗凝治疗以预防血栓栓塞并发症，特别是在有血管受累的患者中。对于有残留疾病的患者，可考虑使用芳香化酶抑制剂等激素治疗。先进的成像技术，包括磁共振成像、计算机断层扫描和超声心动图，在术前规划和术后监测中都至关重要，以确保最佳手术策略并帮助监测残留肿瘤。多学科协作在静脉内平滑肌瘤病的管理中至关重要，确保采用全面方法以优化患者预后。
