原发性下腔静脉平滑肌肉瘤19例临床分析

目的探讨原发性下腔静脉平滑肌肉瘤的诊断与治疗。方法回顾分析解放军总医院近二十年来19例原发性下腔静脉平滑肌肉瘤患者的临床资料,并总结其诊断和治疗经验。结果 19例症状各异,但以腰背痛、右上腹痛及双下肢水肿为多见。根据B超、CT或核磁共振（MRI）以及下腔静脉血管造影为主要诊断依据,但要明确肿瘤来源及性质需病理学检查。12例患者完整切除肿瘤,8例结扎下腔静脉,4例重建下腔静脉,其中3例单纯修补重建,1例聚四氟乙烯补片修补重建;完整手术切除患者平均生存时间（49±40）个月,无法切除患者平均生存时间仅（11±6）个月。结论完整切除肿瘤是原发性下腔静脉肉瘤最有效的治疗方法,侧枝循环代偿充分时下腔静脉重建并非必要。

Primary leiomyosarcoma of inferior vena cava:a clinical analysis of 19 cases

Objective To study the diagnosis and treatment of primary leiomyosarcoma of inferior vena cava（PIVCLS）.Methods The clinical data about 19 PIVCLS patients admitted to our hospital from 1995 to 2012 were retrospectively analyzed with its diagnosis and treatment experience summarized.Results The symptoms of the 19 patients were different,mainly manifested as low back pain,right upper abdominal pain and lower extremity edema.PIVCLS was diagnosed mainly depending on the positive findings of B mode ultrasonography,CT,MRI and inferior vena cavography.However,its origin and nature should be identified by biopsy.PIVCLS was removed in 12 patients.The inferior vena cava was ligated in 8 patients and reconstructed in 4 patients（with simple repair in 3 and with polytetrafluoroethylene patch in 1）.The average survival time was significantly longer in patients with their PIVCLS completely removed than in those with their PIVCLS not removed（49±40 months vs 11±6 months）.Conclusion Total resection is an effective procedure for PIVCLS.It is not necessary to reconstruct the inferior vena cava when its collateral circulation is good.