睾丸原发弥漫性大B细胞淋巴瘤5例  

Five cases of primary testicular diffuse large B-cell lymphoma

作　　者：杜少静 刘悦 卢晓红 张轶华[1] 张立英 DU Shaojing;LIU Yue;LU Xiaohong;ZHANG Yihua;ZHANG Liying(Department of Pathology,Beijing Daxing District People’s Hospital,Beijing 102600,China)

机构地区：[1]北京市大兴区人民医院病理科,北京102600

出　　处：《临床与病理杂志》2023年第10期1888-1894,共7页Journal of Clinical and Pathological Research

摘　　要：目的:探讨睾丸原发弥漫性大B细胞淋巴瘤(primary testicular diffuse large B-cell lymphoma,PT-DLBCL)的临床病理特点、诊断与鉴别诊断。方法:通过回顾性分析2013至2022年间北京市大兴区人民医院收治的5例PT-DLBCL患者的临床病历资料,并进行文献复习。结果:5例患者年龄为56~77岁,主要临床表现为无痛性睾丸肿大。病变部位左侧4例(80%),右侧1例(20%);肿瘤最大径为1.3~11.0 cm,所有患者均行根治性睾丸切除术,其中4例为非生发中心B细胞(non-germinal center B cell,non-GCB)型,1例为生发中心B细胞(germinal center B cell,GCB)型。免疫组织化学示肿瘤细胞均表达CD20、PAX5等B细胞标志,Ki-67增殖指数为80%~90%。3例呈c-MyC/B细胞淋巴瘤(B-cell lymphoma,Bcl)-2/Bcl-6三重表达模式。髓样分化因子88(myeloid differentiation factor 88,MyD88)蛋白表达率为100%。Ann Arbor I期2例、II期1例、IV期2例。国际预后指数(International Prognostic Index,IPI)1~2分2例,3分3例。术后4例患者行化学治疗(以下简称“化疗”),1例未行放射治疗(以下简称“放疗”)和化疗。术后随访6~68个月,其中3例死亡,2例生存。结论:PT-DLBCL较为少见,预后较差,多发生于老年男性,治疗以根治性睾丸切除为主,辅以“环磷酰胺+阿霉素+长春新碱+泼尼松(cyclophosphamide+adriamycin+vincristine+prednisone,CHOP)或利妥昔单抗+CHOP(rituximab-CHOP,R-CHOP)”方案化疗,或鞘内注射化疗药物预防中枢神经系统复发。确诊依赖于病理学检查,组织学需与睾丸精原细胞瘤、胚胎性癌等鉴别。Objective:To investigate the clinicopathological features,diagnosis and differential diagnosis of primary testicular diffuse large B-cell lymphoma(PT-DLBCL).Methods:The medical records of 5 cases with PT-DLBCL treated in Beijing Daxing District People’s Hospital from 2013 to 2022 were retrospectively analyzed,and the relevant literatures were reviewed.Results:The ages of the 5 patients ranged from 56 to 77 years,and the main clinical manifestation was painless enlargement of the testis.The lesions were located on the left side in 4 cases(80%),and the right side in 1 case(20%).The maximum tumor diameter ranged from 1.3 to 11.0 cm.All patients underwent radical orchidectomy,with 4 cases being non-germinal center B cell(non-GCB)type and 1 case being germinal center B cell(GCB)type.Immunohistochemistry showed that tumor cells expressed B-cell markers such as CD 20 and PAX 5,and the Ki-67 proliferation index was 80 to 90%.Three cases showed a c-MyC/B-cell lymphoma(Bcl)-2/Bcl-6 triple-expressing pattern.The expression rate of myeloid differentiation factor 88(MyD 88)protein was 100%.According to the Ann Arbor staging,2 cases were stage I,1 case was stage II,and 2 cases were stage IV.The International Prognostic Index(IPI)scores were 1 to 2 in 2 cases,and 3 in 3 cases.After surgery,4 patients received chemotherapy,1 patient did not receive radiotherapy or chemotherapy.The postoperative follow-up ranged from 6 to 68 months,during which 3 patients died,and 2 patients survived.Conclusion:PT-DLBCL is relatively rare,with a poor prognosis,and it mainly occurs in elderly males.Treatment is primarily radical orchiectomy,supplemented by“cyclophosphamide+adriamycin+vincristine+prednisone(CHOP)or rituximab-CHOP(R-CHOP)”regimen chemotherapy or intrathecal injection of chemotherapy drugs to prevent central nervous system recurrence.Diagnosis depends on pathological examination,and histological differentiation from spermatogastoma and embryonic carcinoma of testis.

关 键 词：原发性睾丸淋巴瘤 弥漫性大B细胞淋巴瘤 病理诊断 髓样分化因子88 

分 类 号：R737.21[医药卫生—肿瘤]