A rare case of intravenous leiomyomatosis with metastasis to the circulatory tract: case report and literature review

Intravenous leiomyomatosis (IVL) is a rare condition characterized by the proliferation of uterine smooth muscle cells within the lumen of vascular vessels. Despite its histologically benign nature, IVL can invade cardiac vessels, leading to severe complications such as circulatory failure and heart blockade. This underscores the critical need for accurate diagnosis and comprehensive preoperative management to prevent fatal outcomes. : RESULTS: A previously healthy 41-year-old woman was admitted to Gynecology Department following abnormal findings on a routine abdominal ultrasound (USG). The USG revealed a pelvic tumor accompanied by extensive thrombosis of the ovarian veins and inferior vena cava (IVC). Abdominal magnetic resonance imaging (MRI) showed an enlarged myomatous uterus measuring 85 x 70 x 60 mm with two oval-shaped focal lesions near the left ovary, measuring 50 x 50 x 45 mm and 80 x 50 x 60 mm. Additionally, vascular CT revealed a free-floating thrombus extending from the ovarian veins, right common iliac vein (CIV), IVC, and into the right atrium. The patient was scheduled for a single-step surgery. She underwent a total abdominal hysterectomy (TAH) with bilateral salpingo-oophorectomy (BSO), followed by sternotomy with extracorporeal circulation (ECC) to remove the tumor from the circulatory tract. Postoperative histopathological examination confirmed the diagnosis of IVL. : CONCLUSION: Patients with IVL extending into the circulatory system should be managed by multidisciplinary teams comprising oncological gynecologists, as well as vascular and cardiothoracic surgeons. This collaborative approach ensures radical surgical resection, which is essential in preventing future recurrences of IVL.