Chang Yih-Leong, Lin Chien-Yu, Wang Shoei-Shen, Kuo Kuan-Ting, Lee Yung-Chie, Wu Chen-Tu
Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan.
Clin Transplant. 2002 Dec;16(6):461-4. doi: 10.1034/j.1399-0012.2002.02047.x.
Primary cardiac tumours are rare, with only one quarter of the patients being malignant. The vast majority of malignant neoplasms of the heart are sarcomas. We describe a patient of primary cardiac rhabdomyosarcoma presented as coronary artery disease and recurrent myocardial infarction. Histopathologic finding of the excised native heart revealed a high grade pleomorphic rhabdomyosarcoma in the myoepicardial portion of the anterior wall with rupture. The accompanying unusual feature was myocardial infarction because of tumour emboli of the left anterior descending and left circumflex coronary arteries. After transplantation, the patient developed mild to moderate acute cellular rejection of the transplanted heart on post-transplantation day 1, 8, and 44, respectively. Unfortunately, he expired on the post-transplantation day 47 because of acute rejection, presenting as concomitant intramyocardial and epicardial lymphocytic vasculitis and multifocal myocardial ischaemia. We found that this uncommon medial lymphocytic vasculitis lesion was mediated by T cells and also by antibody directly against smooth muscle cells of small arteries. The consequence of such immune response would be compromised myocardial oxygenation resulting in allograft failure.
原发性心脏肿瘤很少见，仅四分之一的患者为恶性。心脏的绝大多数恶性肿瘤是肉瘤。我们描述了一名表现为冠状动脉疾病和复发性心肌梗死的原发性心脏横纹肌肉瘤患者。切除的心脏组织病理学检查显示，前壁心肌心包部分有高级别多形性横纹肌肉瘤伴破裂。伴随的不寻常特征是由于左前降支和左旋支冠状动脉的肿瘤栓子导致心肌梗死。移植后，患者分别在移植后第1天、第8天和第44天出现轻度至中度的移植心脏急性细胞排斥反应。不幸的是，他在移植后第47天因急性排斥反应死亡，表现为心肌内和心包淋巴细胞性血管炎以及多灶性心肌缺血。我们发现这种罕见的中层淋巴细胞性血管炎病变是由T细胞介导的，也由直接针对小动脉平滑肌细胞的抗体介导。这种免疫反应的后果将是心肌氧合受损，导致移植失败。
