Chalabreysse Lara, Berger Françoise, Loire Robert, Devouassoux Gilles, Cordier Jean-François, Thivolet-Bejui Françoise
Department of Pathology, Hôpital Louis Pradel, BP Lyon Montchat 69394 Lyon, Cedex 03, France.
Virchows Arch. 2002 Nov;441(5):456-61. doi: 10.1007/s00428-002-0711-0. Epub 2002 Sep 27.
Primary cardiac lymphoma is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium, extremely rare in immunocompetent patients, and more frequent in immunodepressed patients. We present 3 retrospectives cases of primary cardiac lymphoma in immunocompetent patients and review 35 cases reported in the literature. Two patients were adults and one was a child. Primary cardiac lymphoma presented with constitutional symptoms in two cases and superior vena caval syndrome in one case. Diagnosis of a tumor mass was made in all cases by transthoracic echocardiography. Primary cardiac lymphoma arose in the heart right chambers in two cases. Histological diagnoses, obtained after thoracotomy, were diffuse large B-cell lymphoma in two cases, and Burkitt's lymphoma in one case. All three cases received chemotherapy, combined with radiotherapy in one patient. Of our patients, 2 are alive and asymptomatic 12 months and 33 months after diagnosis. In conclusion, diagnosis of primary cardiac lymphoma is difficult due to non-specific clinical manifestations and should be considered in patients with a cardiac mass sometimes with pericardial effusion. It is confirmed using transthoracic echocardiography and magnetic resonance imaging and certified using cytology or open biopsy. The only effective treatment is chemotherapy, but prognosis remains poor.
原发性心脏淋巴瘤是一种结外非霍奇金淋巴瘤，仅位于心脏和/或心包，在免疫功能正常的患者中极为罕见，而在免疫抑制患者中更为常见。我们报告了3例免疫功能正常患者的原发性心脏淋巴瘤回顾性病例，并复习了文献中报道的35例病例。2例为成人，1例为儿童。原发性心脏淋巴瘤2例表现为全身症状，1例表现为上腔静脉综合征。所有病例均通过经胸超声心动图诊断出肿瘤肿块。2例原发性心脏淋巴瘤起源于右心腔。开胸术后获得的组织学诊断，2例为弥漫性大B细胞淋巴瘤，1例为伯基特淋巴瘤。所有3例均接受了化疗，1例患者还接受了放疗。我们的患者中，2例在诊断后12个月和33个月时存活且无症状。总之，由于临床表现不具特异性，原发性心脏淋巴瘤的诊断较为困难，对于有心脏肿块且有时伴有心包积液的患者应考虑此病。通过经胸超声心动图和磁共振成像得以确诊，并通过细胞学检查或开放活检加以证实。唯一有效的治疗方法是化疗，但预后仍然很差。
