Department of Clinical Pathology, Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH 44195, USA.
Am J Surg Pathol. 2012 Oct;36(10):1527-37. doi: 10.1097/PAS.0b013e31825d53b5.
Cardiac lymphomas are rare, and the spectrum of pathologic features is not well defined. We encountered an unusual case of cardiac lymphoma residing within a presumed thrombus. To place such cases in context, we reviewed all cardiac lymphomas presenting to a large US cardiovascular medicine referral center during a 30-year period. A total of 14 cardiac lymphomas were identified, and these included 6 primary cardiac lymphomas (PCLs) and 8 lymphomas secondarily involving cardiac structures. Upon review, 3 of the PCLs were confirmed to be diffuse large B-cell lymphoma, not otherwise specified, involving the myocardium. The other 3 cases of PCL lacked myocardial invasion and showed lymphoma cells embedded in fibrin thrombus. Acute inflammation was not evident. These lymphomas presented in immunocompetent male individuals and involved either a prolapsed myxomatous mitral valve, a pseudomyxoma from the left atrium, or a thrombus arising in a synthetic aortic root graft. All 3 consisted of large atypical lymphocytes expressing a nongerminal center B-cell immunophenotype. Two cases were positive for Epstein-Barr virus (latency type III), but none demonstrated human herpes virus-8 latent nuclear antigen. No systemic disease was found at presentation or during follow-up. In our experience, fibrin-associated large B-cell lymphoma arising in the heart represents a substantial proportion of PCL. These lymphomas appear to represent an underrecognized variant of diffuse large B-cell lymphoma with favorable outcome. Further study is needed to understand their natural history.
心脏淋巴瘤很少见，其病理特征的范围也没有很好地定义。我们遇到了一个不寻常的心脏淋巴瘤病例，它位于一个假定的血栓内。为了将这些病例置于上下文中，我们回顾了一家美国大型心血管医学转诊中心在 30 年期间出现的所有心脏淋巴瘤。共发现 14 例心脏淋巴瘤，包括 6 例原发性心脏淋巴瘤（PCL）和 8 例继发性累及心脏结构的淋巴瘤。经复查，3 例 PCL 被确认为弥漫性大 B 细胞淋巴瘤，非特指型，累及心肌。另外 3 例 PCL 缺乏心肌侵犯，显示淋巴瘤细胞嵌入纤维蛋白血栓中。没有明显的急性炎症。这些淋巴瘤发生在免疫功能正常的男性个体中，涉及脱垂的粘液瘤二尖瓣、左心房的假粘液瘤或合成主动脉根部移植物中的血栓。所有 3 例均由表达非生发中心 B 细胞免疫表型的大异型淋巴细胞组成。2 例为 EBV（潜伏 III 型）阳性，但均未显示 HHV-8 潜伏核抗原。在初次就诊或随访期间均未发现系统性疾病。根据我们的经验，心脏内纤维蛋白相关的大 B 细胞淋巴瘤构成了 PCL 的很大一部分。这些淋巴瘤似乎代表了一种未被充分认识的弥漫性大 B 细胞淋巴瘤变体，预后良好。需要进一步研究以了解其自然病史。
