原发心脏弥漫大B细胞淋巴瘤5例临床病理分析  

Clinicopathological analysis of primary cardiac diffuse large B-cell lymphoma

作　　者：徐玫芳[1] 赵红 康德勇[1] 冯昌银[1] 邱小文[1] 杨焕星[1] 郑宇辉[1] 杨映红[1] XU Mei-fang;ZHAO Hong;KANG De-yong;FENG Chang-yin;QIU Xiao-wen;YANG Huang-xing;ZHENG Yu-hui;YANG Ying-hong(Department of Pathology,Fujian Medical University Union Hospital,Fuzhou 350001,China;Diagnostic Laboratory Service,Fuwai Hospital,Chinese Academy of Medical Sciences,National Center for Cardiovascular Disease,Beijing 100037,China)

机构地区：[1]福建医科大学附属协和医院病理科,福州350001 [2]国家心血管病中心/中国医学科学院阜外医院实验诊断中心,北京100037

出　　处：《临床与实验病理学杂志》2021年第2期167-171,共5页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨原发心脏弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma, DLBCL)的临床病理特点、诊断、治疗及预后。方法回顾性分析5例原发心脏DLBCL的临床病理资料,分别行HE染色、免疫组化EnVision两步法染色,应用原位杂交以及荧光原位杂交(fluorescence in situ hybridization, FISH)进行检测,并复习相关文献。结果镜下见肿瘤细胞为大淋巴样细胞,弥漫性浸润性生长,核分裂象多见。肿瘤细胞弥漫表达CD20、PAX5,Ki-67增殖指数60%~95%,CD5阳性且C-MYC和BCL-2双表达1例。FISH检测未检出MYC、BCL-2、BCL-6基因断裂分离。2例行肿物切除/活检,未行化疗,分别于术后2、3个月死亡;3例行肿物切除/活检及R-CHOP方案化疗,分别随访2、10、38个月,预后良好。结论原发心脏DLBCL属于罕见的高度侵袭性恶性肿瘤,除特征性的病理形态学改变,需结合临床表现、免疫表型及分子遗传学检测以提高诊断的准确性,并进行规范治疗。Purpose To study the clinicopathological features,diagnosis,therapy and prognosis of primary cardiac diffuse large B-cell lymphoma(DLBCL).Methods Five cases of primary cardiac DLBCL were collected and the clinicopathological data including tumor morphology,staining,immunohistochemical EnVision two-step staining,in situ hybridization and fluorescence in situ hybridization(FISH)were performed,with review of the literatures.Results Histopathologica1ly,the tumor cells were large lymphoid cells with diffuse infiltrating growth and high mitotic activity.The tumor cells were positive for CD20 and PAX5,and the Ki-67 index ranged from 60%to 95%.Tumor cells in one of the five cases were positive for CD5,with double expression of C-MYC and BCL-2.No MYC,BCL-2 or BCL-6 gene rearrangements were detected by fluorescence in situ hybridization.Two patients defaulted chemotherapy after tumor resection/biopsy and died 2 and 3 months after surgery,respectively.While another three patients treated with rituximab,cyclophosphamide,doxorubicin,vincristine and prednisone(R-CHOP)therapy after tumor resection/biopsy,and remained in good condition for 2,10 and 38 months after initial diagnosis respectively.Conclusion Primary cardiac DLBCL is a rare and highly invasive malignant tumor.In addition to the characteristic histomorphology,clinical symptoms,immunophenotypic profiles and molecular genetic detection should be combined to improve the diagnostic accuracy,so as timely and standardized therapy could be conducted accordingly.

关 键 词：心脏肿瘤 弥漫大B细胞淋巴瘤 免疫组织化学 分子特征 诊断 

分 类 号：R732.1[医药卫生—肿瘤]