累及下腔静脉及右心房的子宫静脉内平滑肌瘤病的治疗策略(附18例报道)

Therapeutic Strategy of Intravenous Leiomyomatosis with Intracaval and Intracardiac Extension in 18 Cases

目的总结子宫静脉内平滑肌瘤病的诊断和治疗方法，并比较同期手术及分期手术的优劣。方法回顾性分析北京协和医院2002年1月至2013年9月期间收治的18例累及下腔静脉及右心房的子宫静脉内平滑肌瘤病患者的临床病理资料，比较同期手术及分期手术患者的术中出血量、输血量、手术时间、并发症发生率、ICU住院时间、总住院时间及住院费用。结果18例患者均经术后病理学检查诊断为子宫静脉内平滑肌瘤病。其中行同期手术6例（33．3％），行分期手术12例（66．7％）。同期手术组与分期手术组患者在术中出血量、输血量、手术时间、ICU住院时间、总住院时间及住院费用上的差异均无统计学意义（P〉0．05）。同期手术组术后发生1例胸腔积液，1例喉上神经损伤，1例肺部感染；分期手术组发生2例肠梗阻，2例胸腔积液，1例心律失常。2组患者的并发症发生率比较差异无统计学意义（P=1．000）。分期手术组有2例行二期手术时血管内平滑肌瘤向下腔静脉进展，增加了二期手术的难度。术后获访13例，其中同期手术组3例（随访48-63个月，中位随访时间为62．0个月），分期手术组10例（随访1-43个月，中位随访时间为19．5个月）。随访期间，分期手术组复发1例，无死亡病例。结论同期手术与分期手术对子宫静脉内平滑肌瘤病患者来说均是安全和有效的。在患者可以耐受的情况的下，为避免二次手术打击，同期手术对于累及下腔静脉和右心房的子宫静脉内平滑肌瘤病患者是更好的选择。

Objective To summarize the diagnosis and management of intravenous leiomyomatosis, and to compare effect of the one-stage surgery and two-stage surgery. Methods Clinicopathological data of 18 patients hospi- talized in Peking Union Medical College Hospital who were diagnosed as intravenous leiomyomatosis with intracaval and intracardiac extension during Jan. 2002 to Sep. 2013 were collected, and some indexes of the one-stage surgery group and two-stage surgery group were compared, including blood loss, blood transfusion, operation time, period of stay in ICU, hospital stay, and hospitalization expense. Results All the patients were diagnosed as intravenous leiomyomatosis pathologically after operation. Of the 18 patients, 6 （33.3%） patients underwent one-stage surgery and 12 （66. 7%） pati-ents underwent two-stage surgery. There were no significant difference on blood loss, blood transfusion, operation time, period of stay in ICU, hospital stay, and hospitalization expense （P〉0.05）. There were some patients suffered from complication, including 1 case ofpleural effusion, 1 case of recurrent laryngeal nerve injury, 1 case of pulmonary infection in one-stage surgery group; 1 cases ofarrhythrnia, 2 cases of intestinal obstruction, 2 cases ofpleural effusion in two-stage surgery group. No significance had be found in incidence rate of complication between one-stage surgery group and two-stage surgery group （P=I. 000）. Tumors of 2 patients who underwent two-stage surgery had developed before the secondsurgery, increasing the difficulty and risk of the second surgery. Three cases of one-stage group were followed-up for 48-63 month （the median time was 62.0 months）, 10 cases in two-stage group were followed-up for 1-43 month （the median time was 19.5 months）. During the followed-up period, occurrence happened in 1 case of two-stage group, but without death in all cases. Conclusions Both one-stage surgery and two-stage surgery are effective and safe. Taking physical and psychological endurance of patients into consideration, one-stage surgery is highly recommended if the patient is in good status and can tolerate the strike brought by the surgery.