静脉内平滑肌瘤病9例手术治疗临床分析

Clinical analysis of surgery for intravenous leiomyomatosis in 9 cases

目的探讨静脉内平滑肌瘤病(intravenous leiomyomatosis,IVL)的临床特征及治疗方法。方法回顾性分析9例IVL患者的临床资料和术后随访结果。结果9例患者主要症状为胸闷、胸痛、头晕、一过性晕厥或下肢肿胀等,其中4例术前检查提示肿瘤游离端延及心脏;8例术中见肿瘤瘤体根部均位于子宫内,并沿血管走行生长;除1例既往行子宫及双附件切除者外,余8例均行一期肿瘤切除+子宫及双附件切除手术;术中出血量(2147±1275)mL,手术时间(7.6±1.2)h,术后切口愈合良好;术后1例并发不完全性肠梗阻,经保守治疗3 d症状缓解;随访6~12个月,9例均未见复发。结论IVL临床表现缺乏特异性;对术中发现肿瘤根部源自子宫并沿血管走形生长的IVL,一期手术切除肿瘤+子宫及双附件疗效确切;对累及心脏者应尽早摘除瘤体,以恢复正常血流动力学。

Objective To investigate the clinical characteristics and treatment of intravenous leiomyomatosis(IVL).Methods The clinical data and follow-up result were retrospectively analyzed in 9 patients with IVL.Results The main symptoms of 9 patients were chest tightness,chest pain,dizziness,chest pain,transient syncope and lower limb swelling.The preoperative examination showed the free end of the tumor extended to the heart in 4 patients.The surgery presented the root of the tumor was located in the uterus,growing along the vessel in 8 patients.Eight patients underwent one-stage tumor resection plus hysterectomy and double adnexal resection except 1 patient with previous hysterectomy and double adnexal resection.The intraoperative blood loss was(2147±1275)mL,the operation lasted(7.6±1.2)h,and the incision healed well.Incomplete intestinal obstruction occurred in 1 patient after surgery,and was relieved after conservative treatment for 3 days.The 60 to 12-month follow-up showed no recurrence.Conclusion The clinical manifestation of IVL is not specific.One-stage tumor resection plus hysterectomy and double adnexal resection is effective for those with the root originating from the uterus and growing along the vessel.For the patients with heart involvement,the tumor should be removed as soon as possible to restore normal hemodynamics.