静脉内平滑肌瘤病延伸至右侧心腔5例报告

Diagnosis and Surgical Treatment for Intravenous Leiomyomatosis with Intracardiac Extension (Report of 5 Cases)

目的:探讨静脉内平滑肌瘤病延伸至右侧心腔的诊断与外科治疗。方法:2001年1月至2009年1月我院收治静脉内平滑肌瘤病延伸至右侧心腔患者5例,年龄41～49岁,心功能(NYHA)Ⅲ级3例,Ⅱ级2例。均为女性,5例患者均有不同程度的心慌、胸闷、晕厥症状,病程4天～5个月。体检示双下肢水肿、腹水及肝大2例,盆腔占位3例。影像学检查提示,5例患者下腔静脉、右心占位。3例有因子宫肌瘤行子宫切除术病史,1例有左卵巢畸胎瘤剔除史;腹部手术与本次手术间隔15～36个月。肿瘤起源于左右生殖静脉各1例,起源于左髂内静脉2例,右髂内静脉1例。术前行输尿管DJ管植入术2例。结果:2例患者接受分期手术。3例患者一期行盆腔肿瘤及体外循环下行下腔静脉、右心肿瘤切除手术,2例停循环时间分别为29,43min。无围手术期并发症,术后随访1～72个月,心功能明显改善,其中Ⅰ级4例,Ⅱ级1例,1例术后3个月B超发现盆腔复发肿瘤,随访5年无盆腔包块明显增大,患者无自觉症状。3例经下腔静脉切开的患者,1例行下腔静脉重建,其它2例均直接修补,术后随诊无下腔静脉梗阻。结论:提高对有子宫肌瘤病史或右心房肿物患者的警惕性,外科直视下进行心脏内肿物子宫附件病变组织及盆腔残余瘤体切除术,可获满意的外科治疗效果。

Objective： To summarize the diagnosis and surgical treatment of intravenous leiomyomatosis with right intracardiac extension in 5 patients. Methods： From January 2001 to January 2009, 5 female patients diagnosed with intravenous leiomyomatosis with intracardiac extension underwent surgical treatment using circulatory bypass, with single-staged surgical＇resection in 3 patients and two-staged surgical resection in 2 patients. The total circulatory arrest time in the 2 patients was 29 and 43 minutes. Physical examination revealed severe congestive failure with signs of hepatomegaly, ascites or lower extremity edema in 2 patients. A pelvic mass was present in 3 cases. Four patients had a history of laparotomies due to uterine leiomyoma or gonadal teratoma, with an interval of 15 to 36 months between the performance of lapratomy and the removal of the IVL. Imaging findings showed intracaval and intracardiac masses in all 5 patients, originating from the left gonadal vein in 1 patient, right gonadal vein in 1 patient, the left internal lilac vein in 2 patients and the right internal Uiac vein in 1 patient. Prior to surgery, the 2 patients with tumors originating from the gonadal veins received the placement of ureteral double J stents into the ipsilateral ureters. Results： Of the 5 patients, 2 patients underwent successful two-stage surgical removal of IVL and 3 patients had complete one-stage surgical treatment, with 2 patients under deep hypothermic circulatory arrest. The total circulatory arrest time was 29 and 43 minutes in the 2 patients. All patients were followed up for 1-72 months after surgery. All patients had uneventful recoveries and no deaths occurred. Four patients were doing well with no signs of recurrence. A pelvic mass was found by ultrasound in the fifth patient at 3 months after surgery. This patient had experienced no symptoms and no intravenous mass in spite of rejecting any treatment. Three patients received hormone therapy. No obstruction occurred in the 3 patients with inferior cava ventomy. Postoperative NYHA classification was I in 3 cases and II in 2 cases. Conclusion： Leiomyomatosis should be suspected among patients with neoplasm in the right atrium and a history of hysteremyoma. Successful therapy for intravenous leiomyomatosis is dependent on open heart surgical excision of the tumor and laparotomy including hysterectomy, salpingo-oophorectomy and removal of residual or recurrent pelvic mass.