Department of Cellular Pathology, James Cook University Hospital, Marton Rd, Middlesbrough, TS4 3BW, United Kingdom.
Pathol Res Pract. 2012 Mar 15;208(3):172-6. doi: 10.1016/j.prp.2011.12.001. Epub 2012 Feb 10.
Cardiac myxoma and diffuse large B-cell lymphoma are uncommon tumors, yet four composite tumors have been reported since 2009. We are reporting on the fifth case providing detailed immunohistochemical and FISH analyses. The lymphoma was present as superficially located nests of large cells with patchy necrosis in the background of a typical atrial myxoma. It displayed features of DLBCL with non-germinal center phenotype, expressed EBER, LMP1, EBNA2 and shared the following features with the previously reported cases: B-cell lineage, high-grade cytology, high proliferation rate, EBV infection in latency type 3 with one tested case and an excellent outcome. The lymphomas arising within myxoma may follow a pathogenic pathway driven by EBV, whose transformation potential is unleashed in the cytokine-rich milieu of a myxoma, presumably accentuating age-related decline of adaptive immunity known as immune senescence. DLBCL arising within atrial myxoma grouped together with EBV+ DLBCL associated with valve prosthesis and with an atrial thrombus differs in the immunocompetent patients from primary cardiac DLBCL, not otherwise specified, in clinical presentation, pathological features and a course of the disease. Distinction between these groups may have important therapeutic consequences.
心脏黏液瘤和弥漫性大 B 细胞淋巴瘤是不常见的肿瘤，但自 2009 年以来已经报告了四例复合肿瘤。我们报告了第五例病例，提供了详细的免疫组化和 FISH 分析。淋巴瘤表现为位于表面的大细胞巢，背景为典型的心房黏液瘤，伴有斑片状坏死。它表现为非生发中心表型的弥漫性大 B 细胞淋巴瘤特征，表达 EBER、LMP1、EBNA2，并与之前报告的病例具有以下共同特征：B 细胞谱系、高级别细胞学、高增殖率、潜伏类型 3 的 EBV 感染，其中一个病例的预后良好。在黏液瘤中发生的淋巴瘤可能遵循由 EBV 驱动的致病途径，其转化潜能在黏液瘤富含细胞因子的环境中释放，可能会加剧已知的适应性免疫功能下降，即免疫衰老。在免疫功能正常的患者中，发生在心房黏液瘤中的弥漫性大 B 细胞淋巴瘤与 EBV+ 弥漫性大 B 细胞淋巴瘤与瓣膜假体和心房血栓相关，与非特指的原发性心脏弥漫性大 B 细胞淋巴瘤在临床表现、病理特征和疾病过程中有所不同。区分这些组可能具有重要的治疗意义。
