心房黏液瘤伴纤维素相关弥漫性大B细胞淋巴瘤六例临床病理学特征  

Clinicopathological features of fibrin-associated diffuse large B cell lymphoma arising within concurrent atrial myxoma: report of six cases

作　　者：严金海 罗东兰[1] 张芬[1] 陈玉[1] 骆新兰[1] 许洁[1] 李智[1] 刘艳辉[1] Yan Jinhai;Luo Donglan;Zhang Fen;Chen Yu;Luo Xinlan;Xu Jie;Li Zhi;Liu Yanhui(Department of Pathology,Guangdong Provincial People′s Hospital/Guangdong Academy of Medical Sciences,Guangzhou 510080,China)

机构地区：[1]广东省人民医院广东省医学科学院病理医学部病理科,广州510080

出　　处：《中华病理学杂志》2020年第10期1027-1030,共4页Chinese Journal of Pathology

基　　金：国家临床重点专科建设项目;广东省自然科学基金(2019A1515011643)。

摘　　要：目的探讨心房黏液瘤伴纤维素相关弥漫性大B细胞淋巴瘤的临床病理学特征、治疗及预后情况。方法收集广东省人民医院2006-2019年诊断的6例心房黏液瘤伴纤维素相关弥漫性大B细胞淋巴瘤的临床病理信息,分析其组织学形态、免疫表型、分子特征、治疗及预后情况。结果患者年龄46~78岁,平均年龄59岁,男女各3例,均因为左心房黏液瘤就诊。病理检查偶然发现在黏液瘤周边可见大的异型淋巴样细胞弥散分布,可见核分裂象及凋亡细胞,少数坏死。免疫表型为B细胞性(CD20+,CD79α+)、高增殖指数(Ki-67阳性指数>85%)和潜伏Ⅲ型(LMP1、EBNA2、EBER均阳性)的EB病毒感染模式。肿物均行外科手术完整切除,且未做术后化疗。经随访5~120个月,所有患者情况良好,未见复发或转移。结论心房黏液瘤伴纤维素相关弥漫性大B细胞淋巴瘤是一种特殊类型的慢性炎性病变相关弥漫性大B细胞淋巴瘤,表现为惰性的临床病程。手术完整切除肿物,术后密切随访,暂不行化疗以免过度治疗。Objective To investigate the clinicopathological features,treatment and prognosis of fibrin-associated diffuse large B cell lymphoma(DLBCL)arising within concurrent atrial myxoma.Methods Six cases of fibrin-associated DLBCL arising within concurrent atrial myxoma diagnosed at the Department of Pathology,Guangdong General Hospital,from 2006 to 2019 were included.The histology,immunophenotype,treatment and prognoses were analyzed.Results The patients′age ranged from 46 to 78 years(mean 59 years).There were 3 males and 3 females.The tumors were all discovered incidentally on histological examination of surgical pathology specimens excised for atrial myxoma.All patients appeared to have morphological features of DLBCL,B lineage immunophenotype,high proliferative index and latency type III of Epstein-Barr viral infection.They had complete tumor resections without adjuvant chemotherapy and were healthy at 5-to 120-month follow-ups.Conclusions Fibrin-associated DLBCL arising within concurrent atrial myxoma is an unusual form of DLBCL associated with chronic inflammation,and its clinical outcome is indolent.The findings suggest that this type of lymphoma does not warrant excessive or unnecessary treatments after complete resection.

关 键 词：心脏肿瘤 黏液瘤 纤维素 淋巴瘤 大B-细胞 弥漫性 疱疹病毒4型 人 

分 类 号：R737.33[医药卫生—肿瘤]