Overcoming the diagnostic challenges in a high-risk invasive primary cardiac lymphoma.
Overcoming the diagnostic challenges in a high-risk invasive primary cardiac lymphoma.
复制标题
DOI：
10.1093/ehjci/jez324
复制
发表时间：
2020-01
期刊：
影响因子：
--
通讯作者：
Stephani C. Wang;Chris W. Pan
中科院分区：
文献类型：
--
作者：
Stephani C. Wang;Chris W. Pan
原发性心脏淋巴瘤是一种罕见的疾病，其诊断和治疗具有挑战性。一位58岁的男性患者因急性胸痛和呼吸急促被送到急诊室，并被发现处于完全性心脏传导阻滞状态。住院前几个月诊断为心包填塞需要紧急心包窗、马尾综合征和血小板减少症的病史具有重要意义。尽管进行了广泛的检查，包括心包液细胞学检查呈阴性，但病因尚不清楚。经胸超声心动图显示新的右心房肿块，复发性心包积液。随后的经食管超声心动图（TOE）显示弥漫性心内/心包肿块（图A）。心脏磁共振成像（MRI）显示心外膜、心内、心肌内和房间隔区域广泛肿块（*）（图B）。肿块侵入心外膜房室，包绕右冠状动脉（白色箭头）和左回旋支（黑色箭头）（图C）。此外，前内侧面有一个沿着肿块紧靠心包衬里。冠状动脉计算机断层扫描（CT）血管造影证实了上述结果。组织诊断受到严重难治性血小板减少症的挑战。最后，放置临时经静脉起搏器，然后对邻接心包衬里的肿块进行左侧机器人辅助经胸活检。最终细胞学检查显示原发性心脏大B细胞淋巴瘤。然后开始化疗（EPOCH-R），治疗后3天，重复TOE显示肿瘤负荷显著消退（图D）。这是一个罕见的情况下，浸润性原发性心脏淋巴瘤完全心脏传导阻滞，难治性血小板减少症，副肿瘤马尾综合征。这个复杂的病例强调了多种诊断方式的重要性，包括超声心动图，冠状动脉CT血管造影，心脏MRI以及微创组织活检在诊断心脏肿瘤中的作用。
Primary cardiac lymphoma is uncommon and challenging to diagnose and treat. A 58-year-old man presented to the emergency room with acute chest pain and shortness of breath and was found to be in complete heart block. Medical history was significant for cardiac tamponade requiring emergent pericardial window, cauda-equina syndrome, and thrombocytopenia diagnosed several months prior to the admission. Aetiologies were un clear despite extensive work-up, including negative pericardial fluid cytology. Trans thoracic echocardiogram revealed new right atrial masses, recurrent pericardial effusion. Subsequent transoesophageal echocardiogram (TOE) disclosed diffuse intra/pericardial masses (Panel A). Cardiac magnetic resonance imaging (MRI) revealed extensive masses (*) in epicardiac, intracardiac, intramyocardial, and interatrial septal regions (Panel B). The masses invaded epicardial atrioventricular, encasing the right coronary artery (white arrow) and left circumflex artery (black arrow)(Panel C). In addition, there was a mass along the anterior medial surface abutting the pericardial lining. Coronary computed tomography (CT) angiogram reinforced above findings. Tissue diagnosis was challenged by severe refractory thrombocytopenia. Eventually, a temporary transvenous pacemaker was placed, followed by left robotic assisted transthoracic biopsy of the mass abutting the pericardial lining. Final cytology showed primary cardiac large B-cell lymphoma. Chemotherapy (EPOCH-R) was then initiated, and 3 days after treatment, a repeat TOE showed significant regression in tumour burden (Panel D). This was a rare case of an invasive primary cardiac lymphoma with complete heart block, refractory thrombocytopenia, and paraneoplastic cauda-equina syndrome. This complex case highlights the significance of multiple diagnostic modalities including echocardiogram, coronary CT angiogram, and cardiac MRI in addition to minimally invasive tissue biopsy in diagnosing cardiac tumours.