Intravenous Leiomyomatosis:A Study Of 20 Cases And Literature Review

BackgroundIntravascular leiomyomatosis(IVL)is a rare kind of mesenchymal tumor,which is histologically benign but malignant on biological behavior.In some cases the tumor may extend to inferior vena cava,right heart and pulmonary artery,even cause sudden death.Clinical manifestations of IVL are various and nonspecific,even partial patient do not have any clinical symptom.IVL has no specific serum tumor markers.Early diagnosis of IVL is difficult and the rate of misdiagnosis is high.Because of the low incidence of IVL,it has been reported mostly in the form of case reports and case cohort reports.ObjectiveTo explore the epidemiology,clinical features,diagnosis,pathology,treatment and prognosis of IVL.MethodsThe clinical data of all cases of IVL confirmed by pathology in Qilu Hospital,Shandong University since January 1,2007 to March 31,2019 were screened Relevant literature was retrieved from PubMed literature database and a total of 274 IVL cases from 215 case reports were included.Clinical data of all included IVL patients were summarized and analyzed.SPSS 23.0 software was used for data processing and statistical analysis.K-S test was performed on the data.The data in line with the normal distribution were represented by the mean,while the data not in line with the normal distribution were represented by the median(quartile spacing).The chi-square test was used to compare the categorical variables.Kaplan-meier plot was adopted to characterize the progression free survival(PFS)curve of each variable,and possible meaningful variables were integrated into the Cox regression model to analyze the multifactor survival.Results1.A total of 20,436 cases of uterine fibroids were admitted to Qilu Hospital of Shandong University from January 2007 to February 2019,among which 20 cases were diagnosed as IVL by pathology,accounting for 0.97‰ of all cases of leiomyoma.IVL accounted for 0.97‰ of all leiomyoma cases.A total of 274 IVL cases from 215 case reports were included.2.75.9%of IVL patients were multipara.The age ranged from 21 to 81 years old,and the median age(P255 P75)was 46(40,51)years old.There was no significant difference in survival analysis between IVL patients aged<45 years and IVL patients aged>45 years(P=0.360).3.33.9%of IVL patients had a previous history of uterine fibroids,and 22.6%had a previous history of hysterectomy.However,the history of hysterectomy did not affect the prognosis(P=0.213).4.The median diameter(P25,P75)of intravenous tumors was 18.0(6.0,33.5)cm.Intravenous tumor size was not a prognostic factor for IVL(P=0.445).5.There was no statistical difference in survival analysis among IVL patients with different stages(P=0.323).6.The positive rate of CA125 was 21.2%,while it was not the same in IVL patients at different stages.The positive rate of CA125 in stage I and II patients was 11.8%,lower than that in stage III and IV patients(31.3%),but the difference was not statistically significant(P=0.225).Elevated CA125 did not affect the prognosis(P=0.452).7.Bilateral oophorectomy(P=0.045)and complete tumor resection(P=0.003)are both independent risk factors for the prognosis of intravenous leiomyomatosis,while one-stage/two-stage surgery(P=0.900)and endocrine therapy(P=0.883)after surgery have no significant effect on the prognosis.Conclusion1.IVL is a rare benign smooth muscle tumor,which accounted for 0.97‰ of all leiomyoma cases.2.Clinical manifestations of IVL are various and nonspecific,even partial patient do not have any clinical symptom.IVL has no specific serum tumor markers and is extremely easy to be missed before extending to the inferior vena cava.Strengthening intraoperative and postoperative examination of intrauterine and periuterine veins is helpful to improve the early diagnosis rate.3.Women with intravenous occupying should be alert to IVL if they have uterine fibroids or a history of uterine fibroids surgery.4.Surgery is the preferred treatment for IVL.Bilateral oophorectomy and complete tumor resection can improve the prognosis of IVL patients,help to reduce the recurrence rate and prolong the recurrence interval.