Individualized surgical treatment for intravenous leiomyomatosis with extension to the right heart

doi:10.3969/j.issn.1672-5301.2017.10.014

Abstract：Objective Controversy exists regarding surgical treatment for intravenous leiomyomatosis with the right cardiac extension (IVLC).This study aimed to report the results of management of the tumor at a single center.Methods We reviewed 41 patients with removal of IVLC from November 2002 to March 2017.Clinical features,surgical techniques,operative outcomes and available follow-up data were analyzed,and the results were compared with the available English literature.Statistical analyses were carried out by means of Fisher exact test and Student's t test.Results All 41 patients underwent removal of IVLC.The mean age was (44.6±6.2)years (range 25 to 55 years).The clinical presentations were as follows:22 (53.7％) cases of dyspnea,chest tightness and shortness of breath,13(31.7％) of double lower limb edema,4(9.8％) of syncope,4(9.8％) of serous cavity effusion,32(78％)of pelvic mass,4(9.8％)of pulmonary embolism,3(7.3％)of pulmonary metastasis and 10 (24.4％) of lack of symptom.All the patients had history of uterine leiomyoma,30(73.2％) patients had previous hysterectomy/myomectomy and 2 patients had previous fibroid by cardiotomy.15 (36.6％) cases had origin of iliac vein,19(46.3％) cases origin of ovarian vein and 6(14.6％) cases both origin of both veins.Of the 41 cases with ICVL,35(85.4％) cases underwent removal of IVCL under CPB,32(78.0％) cases under DHCA.Staged procedures were performed in 14(41.5％) cases,one-stage procedure in 24(51％) cases.Downward extracting by only laparotomy were performed in 6(14.6％) patients,Upward extraction by only sternotomy in 1(0.3％) patients,removal of tumor by sternolaprotomy with infrahepatic in cavotomy 20 (48.8％) patients,and removal of tumor by sternolaprotomy with retrohepatic cavotomy in 14(34.1％) patients.All 41 patients survived with a smooth postoperative course.The postoperative histology confirmed IVCL.The residual tumors in 9 cases were no of progression in 8 patients with IVCL arising from the iliac vein.During a mean follow-up period of (52.4±36.1)months (range 9-172 months).There was no short or long-term morality.Conclusion We introduce a personalized surgical strategy that provides a way to specify the choice of surgical treatment.This strategy may represent more effective and safe surgical treatment of IVCL.However,larger prospective multi center studies are necessary.