原发性纵隔脂肪肉瘤:单中心研究并文献复习  

Primary mediastinal liposarcoma:Analysis in a single hospital and literature review

作　　者：张钊华 梁朝阳 郭永庆[1,2] 石彬 宋之乙[1,2] 张真榕 邵为朋[1,2] 刘德若 ZHANG Zhao-hua;LIANG Chao-yang;GUO Yong-qing(Peking University China-Japan Friendship School of Clinical Medicine,Beijing 100029,China)

机构地区：[1]北京大学中日友好临床医学院,北京100029 [2]中日友好医院胸外科,北京100029

出　　处：《中日友好医院学报》2020年第6期335-338,共4页Journal of China-Japan Friendship Hospital

摘　　要：目的:总结原发性纵隔脂肪肉瘤的临床特征、治疗方法及预后。方法:回顾我院2011年2月~2016年6月行手术治疗的5例原发性纵隔脂肪肉瘤患者,并结合文献检索获得的140例中国地区病例资料进行报告。结果:本院5例中男4例、女1例,平均年龄57岁。临床表现以压迫引起的呼吸困难为主。5例患者中高分化型2例、去分化型2例、黏液型1例;均行手术治疗,其中4例完成根治性切除,平均手术时间350min,平均术中出血1060ml。5例患者平均总生存期为33.2个月。3例根治性切除患者出现局部复发,平均无病生存期为27.8个月。结论:原发性纵隔脂肪肉瘤罕见,有症状发现时已体积较大,汇总文献病例:肿瘤平均最大直径17.4cm,误诊率高达67.2%。手术是首选治疗方案,但手术难度较高。根治性手术及病理亚型为高分化型的患者预后较好。Objective:To summarize the clinical features,treatment strategy and prognosis of primary mediastinal liposarcoma.Methods:Five patients with primary mediastinal liposarcoma who underwent surgical treatment in our hospital from 2011 to 2016 and 140 cases based on literature retrieval in Chinese medical institutions are reviewed.Results:There were four males and one female among those 5 patients,with an average age of 57 years.The prominent clinical manifestations were dyspnea caused by compression.Histology showed that two patients were well-differentiated,another two were dedifferentiated,the rest was myxoid.All patients received surgery with an average time of 350 min and an average bleeding of 1060 ml,but only 4 patients’tumors were radically excided.The overall survival was 33.2 months.Local recurrence occurred among three patients underwent radical surgery and the disease-free survival was 27.8 months.Conclusion:Primary mediastinal liposarcoma is rare.Surgery is the first choice of treatment,but the procedure is usually tough.Patients with radical surgery and well-differentiated pathological classification have better prognosis.

关 键 词：纵隔肿瘤 脂肪肉瘤 开胸手术 

分 类 号：R734.5[医药卫生—肿瘤]