延伸至心脏的静脉内平滑肌瘤7例并文献复习

Clinical analysis of intravenous leiomyomatosis with cardiac extension

目的探讨延伸至心脏的静脉内平滑肌瘤的临床特点。方法回顾分析7例心脏受累的静脉内平滑肌瘤患者的临床资料。结果 7例患者均为女性,年龄41～49岁,平均(45±3)岁。其中5例(71%)患者既往因子宫肌瘤行子宫全部或部分切除手术,1例左卵巢畸胎瘤行手术剔除;5例患者分别出现活动后气短、晕厥或肺栓塞症状。超声心动图检查在右心房和(或)右心室内发现肿物,活动度较大,可以堵塞三尖瓣口;肿物均向下腔静脉延续。所有患者均成功接受了静脉和心腔内瘤栓取出术,随诊1例患者盆腔肿瘤复发,余6例患者无复发。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,明确诊断后应立即进行手术。提高对本病的临床表现和影像学认识有助于早期诊断和治疗。

Objective To study the clinical features, diagnosis and treatment of intravenous leimyomatosis （IVL） with cardiac extension. Methods A retrospective analysis was performed on 7 patients of IVL with cardiac extension during the period of 2001-2010 in Peking Union Medical College Hospital. Results All patients were female and the ages were between 41 and 49 years old. 5 patients had a history of hysterectomy and 1 of ovarian embryoma cystectomy. Cardiac involvement typically presented as right-sided congestive symptoms. Syncope or pulmonary embolism was also presented. Echocardiography showed a large, freely mobile and filamentous cast appearing in the right atrium and/or right ventricle. The cast extended from the dilated inferior cave vein into the right heart. All patients underwent surgery successfully. Conclusions IVL with cardiac extension was rare and recognition of the disease will help early diagnosis and treatment.