子宫外脉管内平滑肌瘤病的诊治

脉管内平滑肌瘤病发生与雌激素密切相关。该类子宫肌瘤可以超出子宫范围，可在静脉内延伸达下腔静脉，甚至心脏。由于临床症状多变且不典型，早期诊断十分困难。病变侵及下腔静脉者及右心或肺动脉者可引起充血性右心衰的表现和猝死。此时需要与静脉血栓栓塞症、心房黏液瘤等鉴别。CT和MRI是重要的影像诊断方法。治疗原则是手术彻底切除所有病变，以降低复发率。如病变局限于盆腔，建议行全子宫切除术+盆腔病灶切除术+双侧附件切除，如果累及大血管甚至心腔，完整手术切除病灶需要多学科合作完成。

Diagnosis and treatment of extra-uterus intravenous leiomyomatosis.

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.