原发性下腔静脉平滑肌肉瘤15例诊治分析

Clinical diagnosis and treatment of primary leiomyosarcoma of the inferior vena cava:analysis of 15 cases

目的探讨原发性下腔静脉平滑肌肉瘤的临床特点、影像学特点及病理特点,分析治疗策略及预后。方法回顾性分析2012年6月-2021年7月上海交通大学医学院附属仁济医院泌尿科收治的15例经病理检查证实的下腔静脉平滑肌肉瘤患者的影像学及临床资料,并回顾相关文献。本研究病例中男性3例,女性12例,平均年龄56.3岁。15例患者均行手术治疗。结果15例患者影像学表现为下腔静脉走行区软组织肿块,腔内生长肿瘤强化CT或MRI表现为边缘强化明显,腔外生长肿瘤表现为斑片状不均匀强化;15例手术患者中,15例均完整切除肿瘤,2例切缘阳性,其余切缘阴性;病理均提示为平滑肌肉瘤;中位随访时间33(4~9)个月,中位生存期46个月。结论原发性下腔静脉平滑肌肉瘤临床上罕见,发病早期缺乏特殊症状,预后较差,CT或MRI强化模式在诊断中具有一定意义,手术切除为有效的治疗手段。

Objective To investigate the clinical,imaging and pathological features of primary leiomyosarcoma of the inferior vena cava(PIVCLS),and to analyze the treatment strategy and prognosis.Methods The imaging and clinical data of 15 patients with pathologically confirmed PIVCLS treated during Jun.2012 and Jul.2021 were retrospectively analyzed,and relevant literature was reviewed.There were 3 males and 12 females in this study,with an average age of 56.3 years.All 15 patients underwent surgical treatment.Results The imaging findings of 15 patients were soft tissue masses in the running area of inferior vena cava.CT or MRI showed obvious edge enhancement of intracavitary growth tumor,and patchy,uneven enhancement of extravitary growth tumor.Tumors were completely resected in all 15 cases,with positive margins in 2 cases and negative margins in others.Pathological examination indicated leiomyosarcoma.The median follow-up was 33(4-69)months.The median survival was 46 months.Conclusion PIVCLS is rare in clinical practice,with no special symptoms at the early stage and poor prognosis.Enhanced CT or MRI mode is significant in the diagnosis,and surgical resection is an effective treatment.