纵隔原发性精原细胞瘤二例并国内三十年临床病例资料文献复习

摘要

目的 分析纵隔原发性精原细胞瘤的临床特点、影像学表现及病理表现、治疗及转归等,以期为临床提供参考,提高医生对该病的认识.方法 系统查阅"CNKI、维普网、万方数据知识服务平台"数据库,以"纵隔精原细胞瘤"为关键词对1979年1月-2016年7月发表的中文文献进行检索,通过去重,删除资料不完整等文献后,共纳入资料完整文献59篇,共75例患者,加上本文报道的2例共77例患者.分析其临床表现、确诊方式、影像学表现、病理表现、治疗及预后等资料.结果 77例患者主要为男性(96.1%,74/77),最常见临床症状是胸痛(37.7%,29/77),其次为胸闷(31.2%,24/77);主要的确诊方式是开胸探查术+术后病理检查(80.5%,62/77);影像学表现为纵隔不规则团块影,密度不均匀;增强扫描大部分不均匀强化;病灶位置主要位于前纵隔.病理表现为大部分病灶呈分叶状肿物;光镜下可见成团分布的圆形细胞,瘤细胞呈弥漫片状生长,异形性明显.最常用治疗方法是手术结合放化疗,早期疗效较好,总治愈率为82.4%,总有效率为90.2%,病死率为9.8%,患者死亡的主要原因为全身多处转移.结论 纵隔原发性精原细胞瘤临床少见,多见于青年男性,好发于前纵隔,对放化疗敏感,治疗可手术联合放化疗,早期预后良好,多可治愈.%Objective To analyze the clinical features,imaging and pathological manifestations,treatment and outcome of mediastinal primary seminoma,in order to provide a reference for clinical practice and to improve the doctor′s understanding of the disease.Methods The database of "CNKI,VIP network and Wanfang data"were systemtically consulted.Using "mediastinal seminoma" as the key word,the Chinese literature published from January 1979 to July 2016 was retrieved.By removing duplicates,deleting literature with incomplete data and etc.,a total of 59 studies involving 75 patients were included.Added 2 cases reported in this paper,a total of 77 patients were included.Their clinical features,diagnosis form imaging and pathological manifestations,treatment and prognosis and etc.were analyzed.Results 77 patients were mainly male(96.1%,74/77).The most common clinical symptoms were chest pain(37.7%,29/77),followed by chest distress(31.2%,24/77).The primary form of diagnosis was open chest exploration and postoperative pathology examination(80.5%,62/77).The imaging showed irregular mediastinal mass with uneven density.The enhanced scan was mostly inhomogeneous.The location of the lesion was mainly located in the anterior mediastinum.Pathology showed most lesions showed lobulated mass.Round cells were seen under light microscopy,the tumor cells showed diffuse and patchy growth with obvious atypia.The most commonly treatment was surgery combined with radiochemotherapy.The early curative effect was better.The total cure rate was 82.4%,total effective rae was 90.2%,the mortality rate was 9.8%.The main cause of death was systemic multiple metastasis.Conclusion The mediastinal primary seminoma is rare in clinic and more common in young men.It mainly locates in the anterior mediastinum and is sensitive to radiochemotherapy.The treatment can be surgery combined with radiochemotherapy.The early prognosis is good and can be cured.