子宫静脉内平滑肌瘤病9例临床分析

Clinical analysis of intravenous leiomyomatosis:report of 9 cases

目的探讨子宫静脉内平滑肌瘤病(IVL)临床病理特征、诊断及治疗策略。方法回顾性分析2013年1月至2017年12月浙江大学医学院附属妇产科医院住院手术并经病理证实为子宫IVL且随访完整的9例患者临床资料。结果8702例子宫平滑肌瘤患者中,共诊断9例子宫IVL。患者多因盆腔包块就诊,6例子宫明显增大。术前影像学提示5例存在宫旁包块。除1例合并肺部转移外,所有患者病变均局限于盆腔。除2例行肌瘤剔除术,余患者均行子宫切除术。术中可见4例肌瘤呈树枝状、哑铃形、椭圆形或长条形。9例患者平均随访时间28.2个月,2例复发。结论子宫明显增大和宫旁包块是子宫IVL的重要特征,该病术前诊断困难,术中辨认肌瘤形态特征有助于提高诊断准确性,治疗首选子宫全切术,术后需长期随访。

Objective To investigate the clinical characteristics,diagnosis and treatment of intravenous leiomyomatosis(IVL).Methods The clinical data of 9 IVL treated at Women’s Hospital,Zhejiang University School of Medicine from January 2013 to December 2017 were analyzed retrospectively.All the cases were followed up.Results Out of 8702 patients with uterine leiomyoma,9 patients were diagnosed with IVL.Most patients complained of pelvic mass,and 6 patients had enlarged uterus.Preoperative imaging examination demonstrated parametrial mass in 5 cases.All nine cases were confined to the pelvic cavity except for one who had lung metastasis.Two cases had myomectomy,and seven underwent hysterectomy.The lesions were dendritic,dumbbell-shaped,elliptical or elongate in four cases.Nine cases had a mean follow-up of 28.2 months,and recurrence occurred in two cases.Conclusion The obvious enlargement of uterus and parametrial mass are important features of IVL.It is difficult to diagnose preoperatively.Identifying the morphological features of lesions during operation can improve the diagnostic accuracy.Total hysterectomy is the first choice of treatment and long-term follow-up is also needed.