子宫静脉内平滑肌瘤病48例临床分析

Clinical Analysis of 48 Cases of Uterine Intravenous Leiomyomatosis

目的:探讨子宫静脉内平滑肌瘤病(IVL)的临床特征、诊断及治疗方法。方法:回顾性分析中国医科大学附属盛京医院2011年12月—2020年3月收治的48例IVL患者的临床资料,并进行随访。结果:48例患者年龄34~71岁,平均年龄(46.31±7.12)岁,临床表现主要为经量增多(22.9%)、阴道不规则出血(12.5%)、下腹痛(12.5%)及尿频(12.5%)。术前盆腔超声检查中仅3例(6.3%)诊断IVL,计算机断层扫描(CT)和(或)磁共振成像(MRI)检查提示IVL 2例(7.4%),子宫肌瘤6例(22.2%),子宫肌瘤变性7例(25.9%),盆腔囊实性占位12例(44.4%)。共35例患者术前检测CA125水平,7例高于正常,余28例均正常。病灶≥5 cm者40例(83.3%),最大达30 cm;质地软者33例(68.8%);病灶位于子宫体部34例(70.8%),宫颈峡部7例(14.6%),阔韧带6例(12.5%),右侧髂内静脉旁1例(2.1%)。手术术式包括病灶切除术8例(16.7%),全子宫切除术20例(41.7%),全子宫+单侧附件切除术5例(10.4%)及全子宫+双附件切除术15例(31.2%)。获得随访的44例患者中位随访时间为38个月,其中2例复发,复发率4.5%,均为经腹全子宫切除者,其中1例病理提示富细胞型,于术后3年复发,且进展至心脏。结论:IVL临床表现无特异性,术前诊断困难,但当辅助检查提示肿瘤为囊实混合性,与子宫分界不清或肌瘤可疑变性,且CA125正常或轻度升高,术中见肿瘤质地较软时,应高度怀疑IVL。手术切除是主要治疗方式,结合患者情况选择适当的手术方式可获得满意的治疗效果,术中应仔细探查,术后需定期随访,尤其是病理提示富细胞型患者。

Objective:To analyze the clinical features,diagnosis and treatment of uterine intravenous leiomyomatosis(IVL).Methods:A retrospective analysis was performed on 48 cases of IVL admitted to Shengjing Hospital Affiliated to China Medical University from December 2011 to March 2020.Follow-up was conducted.Results:The age of the 48 patients ranged from 34 to 71 years old,with an average age of 46.31±7.12 years old.Symptoms included menorrhagia(22.9%),irregular bleeding(12.5%),lower abdominal pain(12.5%)and frequent urination(12.5%),etc.Only 3(6.3%)patients were diagnosed with IVL by pelvic sonography;while 2(7.4%)patients were diagnosed with IVL,6(22.2%)with uterine fibroid,7(25.9%)with uterine fibroid degeneration,and 12(44.4%)with cystic-solid lesion in pelvic cavity by computed tomography(CT)and/or magnetic resonance imaging(MRI).35 patients underwent CA125 test,7 showed abnormal results,28(80.0%)showed normal results.The lesion was greater than or equal to 5 cm in 40 patients(83.3%),with a maximum of 30 cm.Soft texture in 33 cases(68.8%);The lesions were located in the uterine body in 34 cases(70.8%),cervical isthmus in 7 cases(14.6%),broad ligament in 6 cases(12.5%),and next to the right internal iliac vein in 1 case(2.1%).Surgical methods included resection of lesions(16.7%),total hysterectomy(41.7%),total hysterectomy with unilateral adnexectomy(10.4%)and total hysterectomy with bilateral adnexectomy(31.2%).Follow-up data were obtained in 44 patients,the median follow-up time was 38 months,among which 2 had recurrence,with a recurrence rate of 4.5%.All the patients with recurrence underwent laparohysterectomy and 1 of them was pathologically indicated to be cell-rich,relapsed 3 years after surgery and progressed to the heart.Conclusions:Symptoms of IVL were usually nonspecific,and preoperative diagnosis is difficult.However,IVL should be highly suspected when auxiliary examination indicated that the tumor presented as mixed cystic and solid or suspicious degeneration,and CA125 was normal or mildly elevated,and the tumor was found to be soft intraoperative.Surgical resection is the main treatment,select the appropriate operation method can obtain satisfactory therapeutic effect.It is important to adequately examine intraoperatively.Long-term follow-up is important,especially patients with cell abound type.