伴有心脏受累的静脉内平滑肌瘤病临床分析

Clinical analysis of 3 cases of intravenous leiomyomatosis with cardiac extension.

目的:探讨侵入心脏的静脉内平滑肌瘤的临床特点和治疗方法。方法:回顾分析北京协和医院1983年1月至2004年10月收治的心脏受累的静脉内平滑肌瘤患者3例的临床资料。结果:3例患者有心慌、胸闷、晕厥或静脉栓塞等症状。影像学检查在右心房或右心室发现瘤栓。其中,1例2年前因子宫肌瘤行子宫切除术,2例合并有盆腔包块。3例均无肺转移,均接受了静脉瘤栓取出术,其中1例残留心房内瘤栓,1例手术前后接受大剂量孕激素治疗。结论:静脉内平滑肌瘤病的发生率低,侵入心脏的病例更为罕见。治疗以手术为主,抗雌激素治疗可能改善预后。

Objective ：To study the clinical feature, diagnosis and treatment of intravenous leimyomatosis （IVL） with cardiac extension, nethods：A retrospective analysis was performed on 3 patients of IVL with cardiac extension during the period of 1983 - 2004 in Peking Union Medical College Hospital. Results：All 3 patients presented with cardiac symptoms associated with a right atrial mass who also had a pelvic mass or who had previously undergone hysterectomy because of leiomyoma uteri. None of them had pulmonary metastasis. They were all treated with surgical resection in one-stage or two stages within a short interval. One patient had residual tumor in her right atrium. Another one received continuous high dose of progestogen therapy for more than 3 months. Conclusion：IVL is a rare benign smooth muscle tumor. Surgical excision is the most optimum therapy. Meanwhile hormonal therapy may be helpful to improve the prognosis.