关键词: 脉管内平滑肌瘤病, 子宫外, 充血性右心衰, 静脉血栓栓塞症

Abstract:

The development of intravenous leiomyomatosis（IVL）is closely related to estrogen. IVL often extends outside the uterus，reaching the inferior vena cava and even heart.Early diagnosis is very difficult due to its aspecific and subtle clinical symptoms.Its invasion to the inferior vena cava and the right heart chamber or the pulmonary artery may result in signs of right-sided congestive heart failure and sudden death.Differential diagnosis should be made to exclude venous thromboembolism（VTE）and atrial myxoma.Complete excision of pelvic and intravenous leiomyomatosis guarantees favorable outcomes and excellent prognosis with low rates of recurrence. If IVL confines to pelvic，total hysterectomy + bilateral salpingo-oophorectomy+ lesions resection is preferred.Multidisciplinary team is required for extensive resection of IVL extending to inferior venal and the right atrium.

Key words: intravenous leiomyomatosis, extra-uterus, congestive heart failure, venous thromboembolism