静脉内平滑肌瘤病的临床特征与外科治疗策略  

Clinical features and surgical strategies for intravenous leiomyomatosis

作　　者：徐静[1] 张勇[1] 童汉兴[1] 徐德民 陆维祺[1] XU Jing;ZHANG Yong;TONG Han-xing;XU De-min;LU Wei-qi(Department of General Surgery,Zhongshan Hospital,Fudan University,Shanghai 200032,China;Department of Cardiac Surgery,Zhongshan Hospital,Fudan University,Shanghai 200032,China)

机构地区：[1]复旦大学附属中山医院普通外科,上海200032 [2]复旦大学附属中山医院心外科,上海200032

出　　处：《中国临床医学》2019年第3期369-373,共5页Chinese Journal of Clinical Medicine

摘　　要：目的:探讨静脉内平滑肌瘤病(intravenous leiomyomatosis,IVL)的外科规范化治疗策略。方法:回顾性分析2011年7月到2018年9月复旦大学附属中山医院腹部软组织肿瘤多学科团队收治的22例IVL患者的临床资料。结果:22例患者中,1例行分期手术;余21例患者中,不进行CPB、开腹不开胸手术者1例,部分CPB下开腹不开胸手术者9例,完全CPB下胸腹联合切口手术者10例,完全CPB下开腹联合左侧肋间小切口手术者1例。平均手术时间(315±101)min(173~600min),平均出血量(1475±1084)mL(400~9000mL)。围手术期死亡1例;1例术后急性肾功能损伤,1例术后十二指肠瘘,1例术后凝血功能障碍,1例术后下腔静脉内血栓形成,其余患者无术后并发症。术后随访2~87个月,中位随访时间26个月;随访过程中,1例患者出现盆腔复发及肺良性转移,2例盆腔复发,其余患者无复发转移。结论:IVL累及范围广,术前应全面了解肿瘤与静脉及心腔的关系。对于腹盆腔病灶,建议联合全子宫+双附件切除;对于累及心脏的IVL,建议体外循环下一期手术完整切除病灶;静脉内瘤栓在静脉及心腔内活动度大时,可经腹部切口完成手术。Objective: To explore the standardized surgical strategy for intravenous leiomyomatosis. Methods: The clinical data of 22 patients with intravenous leiomyomatosis were retrospectively analyzed in Zhongshan Hospital, Fudan University from July 2011 to September 2018. Results: Among the 22 patients, 1 patient underwent two-stage surgery, and other 21 patients underwent one-stage surgery, among whom, 1 patient underwent laparotomy without CPB, 9 patients underwent laparotomy with partial CPB, 10 patients underwent thoracotomy+laparotomy with total CPB, 1 patient underwent laparotomy combined with left intercostal small incision surgery with total CPB. The operative time was 173 to 600 min ([ 315± 101] min), and the blood loss was 400 to 9 000 mL ([1 475±1 084] mL). There were 1 case of perioperative death, 1 case of acute renal injury, 1 case of duodenal fistula, 1 case of coagulation dysfunction, 1 case of thrombosis in inferior vena cava, and the rest patients had no surgical complications. After 2 to 87 months of follow-up, with the median follow-up time of 26 months, 1 patient had pelvic recurrence and benign lung metastasis, 2 patients had pelvic recurrence, and the rest had no recurrence and metastasis. Conclusions: Intravenous leiomyomatosis involves multiple organs. Comprehensive preoperative examination of the relationship between tumors and veins or cardiac cavity is necessary before the surgery. For abdominal and pelvic lesions, hysterectomy and adnexectomy is recommended. For intracardiac leiomyomatosis, complete excision of the lesion under cardiopulmonary bypass is the best surgical strategy. If there is no adhesion between the tumor and vein, one-stage surgery through an abdominal approach is feasible.

关 键 词：静脉内平滑肌瘤病 体外循环 一期手术 手术策略 

分 类 号：R732.22[医药卫生—肿瘤]