静脉内平滑肌瘤病57例临床分析

Clinical analysis of 57 cases with intravenous leiomyomatosis.

目的探讨静脉内平滑肌瘤病(intravenous leiomyomatosis,IVL)的临床特点、治疗方法和预后。方法回顾性分析郑州大学第一附属医院2013年1月至2023年1月收治的病理确诊为IVL57例患者的临床资料和随访情况。结果患者年龄(45.2±5.5)岁。有子宫肌瘤剔除史11例(19.3%),有子宫切除史6例(10.5%)。常见的临床症状包括:经量增多、经期延长30例(52.6%),腹胀11例(19.3%),心悸、胸闷6例(10.5%),下肢水肿5例(8.8%)。根据肿瘤侵袭范围将IVL分为4期,Ⅰ期:肿瘤局限于盆腔33例;Ⅱ期:肿瘤扩展至腹腔16例;Ⅲ期:肿瘤侵袭至心脏7例;Ⅳ期:肿瘤侵袭至肺动脉1例。全部患者均行手术治疗,术后定期随访,3例患者术后复发,其中1例再次手术。结论IVL好发于生育期和围绝经期女性,缺乏特异性临床症状。治疗方法以手术治疗为主,要根据病变累及范围决定手术方案,术后需定期复查,严密随访。

Objective To explore the clinical characteristics,treatment and prognosis of intravenous leiomyomatosis(IVL).Methods A retrospective analysis was conducted of the clinical data and follow-up of 57 patients with pathologically diagnosed IVL at the First Affiliated Hospital of Zhengzhou University from January 2013 to January 2023.Results The age of 57 patients was(45.2±5.5)years.11 patients(19.3%)had a history of myomectomy and 6 patients(10.5%)had a history of hysterectomy.The common symptoms:30 patients(52.6%)had increased menstrual volume or prolonged menstrual period,11 patients(19.3%)had abdominal distension,6 patients(10.5%)had palpitation or chest tightness,5 patients(8.8%)had lower limb edema.In this study,IVL was graded into four stages according to intravascular tumor progression.StageⅠ:Tumor confined to the pelvic cavity in 33 cases;StageⅡ:Tumor extended into the abdominal cavity in 16 cases;StageⅢ:Tumor extended into the heart in 7 cases;StageⅣ:Tumor extended the pulmonary artery in 1 case.All 57 patients underwent surgical treatment and were under strict follow-u[after surgery.During postoperative follow-up,3 patients showed evidence of recurrence,and one of them had reoperation.Conclusions IVL is a rare disease that usually occurs in reproductive and perimenopausal women with nonspecific manifestations.Surgey is the primary treatment.The surgical plan should be decided by the extent of tumor involvement.A regular postoperative follow-up is extremely essential.