56例伴纵隔大包块淋巴瘤患者的临床特征及预后分析

目的:分析伴纵隔大包块淋巴瘤患者的发病情况、临床表现、治疗效果及预后。方法:回顾性分析2007年2月至2018年1月诊治的56例初诊伴有纵隔大包块患者的临床资料,包括临床特征、分型、分期、疗效及预后。结果:56例患者中,男性25例(45%),女性31例(55%),中位年龄31(17-72)岁。其中≤40岁患者48例(86%)。按病理分型,其中弥漫大B细胞淋巴瘤非特指型(DLBCL-NOS)9例(16.1%),原发纵隔大B细胞淋巴瘤(PMBCL)26例(46.4%),经典型霍奇金淋巴瘤(c HL)17例(30.4%),纵隔灰区淋巴瘤(MGZL)4例(7.1%)。所有患者均采用化疗或化学免疫治疗,中位随访时间54.5(11-149)个月。c HL组中联合受累野放疗(IFRT)10例,完全缓解(CR)10例(58.8%),部分缓解(PR)2例(11.8%),疾病进展(PD)5例(29.4%),治疗过程中有6例患者出现复发/难治而接受自体造血干细胞移植(auto-HSCT)。c HL组1年总生存率(OS)为94.1%,2年OS率为88.2%,5年OS率为67.2%。MGZL组中,联合IFRT 3例,auto-HSCT 1例,CR 2例(50%),PD 2例(50%)。MGZL组1年及2年OS率均为66.7%,5年OS率33.3%。PMBCL组中,联合IFRT 8例,auto-HSCT 7例,CR 22例(84.0%),PR 2例(8.0%),疾病稳定(SD)1例(4.0%),PD 1例(4.0%)。PMBCL组1年及2年OS率均为100%,5年OS率为95.7%。DLBCL-NOS组中,联合IFRT 3例,auto-HSCT 2例,CR 4例(44.5%),PR 2例(22.2%),疾病稳定(SD)1例(11.1%),PD 2例(22.2%)。DLBCL-NOS组1年OS率100%,2年及5年OS率均为77.8%。4组间OS率存在显著性差异(P<0.05),组间比较,PMBCL组生存期显著长于MGZL组(P<0.01)及c HL组(P<0.05),与DLBCL-NOS无显著性差异(P>0.05)。结论:纵隔肿物因其解剖部位独特,常表现为独特的临床特征。c HL、MGZL、PMBCL、DLBCL-NOS等几种原发或伴发纵隔大包块的B细胞起源的淋巴瘤,好发于年轻患者,其临床表现类似,但治疗效果差异显著。在现有治疗条件下,PMBCL的预后优于MGZL及c HL。

Clinical Characteristics and Prognosis of 56 Lymphoma Patients with Mediastinal Masses

Objective:To analyze the incidence,clinical characteristics,treatment efficacy and prognosis of lymphoma patients with mediastinal masses.Methods:The clinical data of 56 lymphoma patients with mediastinal masses treated in our hospital from February 2007 to January 2018 were collected,and the clinical characteristics,typing,staging,treatment efficacy as well as prognosis analyzed retrospectively.Results:Among the 56 patients,male and female were 25(45%)cases and 31(55%)cases,respectively.The median age was 31(17-72)years old,out of them 48(86%)patients aged below 40 years old.According to pathological classification,9(16.1%)cases were diffuse large B cell lymphoma,not otherwise specified(DLBCL-NOS),26(46.4%)cases were primary mediastinal large B cell lymphoma(PMBCL),17(30.4%)cases were classical Hodgkin’s lymphoma(cHL),and 4(7.1%)cases were primary mediastinal grey zone lymphoma(MGZL).All the patients were treated with chemotherapy or immuno-chemotherapy.The median follow-up time was 54.5(11-149)months.In cHL group,10 cases received involved field radiotherapy(IFRT),10(58.8%)cases achieved complete remission(CR),2(11.8%)cases achieved partial remisson(PR),and 5(29.4%)patients experienced progressive disease(PD),6 patients with relapsed/refractory disease received autologous hematopoietic stem cell transplantation(auto-HSCT),the 1,2,and 5 year overall survival(OS)rates of cHL group were 94.1%,88.2%,and 67.2%,respectively.In MGZL group,3 cases combined with IFRT,1 case with auto-HSCT,2(50%)cases achieved CR,2(50%)cases experienced PD,the 1,2,5 year OS rates of MGZL group were 66.7%,66.7%,and 33.3%,respectively.In PMBCL group,8 cases combined with IFRT,7 cases with auto-HSCT,22(84.0%)cases achieved CR,2(8.0%)cases achieved PR,1(4.0%)case was stable disease(SD)and 1(4.0%)case experienced PD,the 1,2 year OS rates of PMBCL group were both 100%,and 5 year OS rate was 95.7%.In DLBCL-NOS group,3 cases combined with IFRT,2 cases with auto-HSCT,4(44.5%)cases achieved CR,2(22.2%)cases achieved PR,1(11.1%)case was stable disease(SD)and 2(22.2%)cases experienced PD,the 1 year OS rate of DLBCL-NOS cohort was 100%,2,and 5 year OS rates were both 77.8%.There was a significant difference of OS rate among these 4 group(P<0.05).In intra-group comparison,OS rate in PMBCL group were significantly longer than both MGZL group(P<0.01)and cHL group(P<0.05),and showed no significant difference from DLBCL-NOS group(P>0.05).Conclusion:Mediastinal masses are characterized by unique clinical features due to their unique anatomical location.Several B cell originated lymphomas including cHL,MGZL,PMBCL and DLBCL-NOS,manifested primary or secondary mediastina involvement,tend to occur in young adults and have similar clinical features.However,their therapeutic response was significantly different.The prognosis of PMBCL is prior to MGZL and cHL.