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目的总结子宫源性静脉内平滑肌瘤病(IVL)的诊断及治疗方法。方法回顾性分析中国医科大学附属第一医院2004年9月至2015年9月收治的7例IVL的临床资料。结果 7例病人中5例行手术治疗,2例行保守治疗。手术病人术后病理学检查均证实为子宫源性平滑肌瘤病。术中见5例病人均累及子宫静脉、髂静脉、卵巢静脉及下腔静脉,累及右心房4例,累及右心室及肺动脉1例。5例病人IVL均来源于子宫肿瘤。随访结果:7例病人均获随访。1例病人术后2个月因呼吸、循环功能衰竭死亡,2例未手术病人分别于出院后3年及4年死亡。1例低级别子宫内膜间质肉瘤病人术后1年复发。结论 IVL是一种少见但高危的疾病,手术复杂、困难,应在临床诊断上予以足够的重视,防止漏诊及误诊。术前应详细制定手术方案。
Objective To summarize the diagnosis and treatment of uterine-derived intravenous leiomyomatosis (IVL). Methods The clinical data of 7 patients with IVL admitted to the First Affiliated Hospital of China Medical University from September 2004 to September 2015 were retrospectively analyzed. Results Of the 7 patients, 5 were treated surgically and 2 received conservative treatment. Postoperative pathological examination of surgical patients were confirmed as uterine-derived leiomyomata. Intraoperative findings in 5 patients involving uterine vein, iliac vein, ovarian vein and inferior vena cava, involving the right atrium in 4 cases, involving the right ventricle and pulmonary artery in 1 case. 5 cases of patients with IVL derived from uterine tumors. Follow-up results: All 7 patients were followed up. One patient died of respiratory failure and circulatory failure 2 months after operation, and two patients died of non-operation 3 and 4 years after discharge respectively. One case of low-grade endometrial stromal sarcoma recurred after one year. Conclusion IVL is a rare but high-risk disease. The surgery is complicated and difficult. It should be given enough attention in clinical diagnosis to prevent misdiagnosis and missed diagnosis. Preoperative surgical plan should be developed in detail.