Ceresoli G L, Ferreri A J, Bucci E, Ripa C, Ponzoni M, Villa E
Department of Radiochemotherapy, Ospedale San Raffaele, Milan, Italy.
Cancer. 1997 Oct 15;80(8):1497-506. doi: 10.1002/(sici)1097-0142(19971015)80:8<1497::aid-cncr18>3.0.co;2-0.
Primary cardiac lymphoma (PCL) is extremely rare in immunocompetent patients. Different definition criteria have been employed in published series. Prognosis is poor due to diagnostic delay and relevance of the site of disease.
Two cases observed at the study institution are reported, with a review of 48 cases published in the literature from 1980 to 1996. Only patients with lymphoma confined to the heart and/or pericardium and those with a single and asymptomatic extracardiac site were considered for analysis.
Eight patients had minimal extracardiac disease. The most common presentation was unresponsive heart failure. Electrocardiography findings were not specific. PCL usually arose in the right chambers as a mass, with or without pericardial effusion (> 80%). Chest X-rays, transthoracic echocardiography, and computed tomography scans are standard in diagnostic workup, but transesophageal echocardiography (TEE) and magnetic resonance imaging (MRI) showed a sensitivity > 90%. Cytology of pericardial effusion was diagnostic in 67% of cases. Thoracotomy was diagnostic in all cases, whereas less invasive procedures had high false-negative rates. Gross resection has no role. Early anthracycline-containing chemotherapy appears to improve survival, whereas the role of radiotherapy has not yet been defined.
The diagnosis of PCL should be considered in patients with a cardiac mass and/or unexplained refractory pericardial effusion. Adequate diagnostic workup, including TEE and MRI, allows confirmation of the early suspicion of PCL. In the absence of a diagnostic cytology, an open biopsy may be indicated to avoid treatment delay. There is no evidence that PCL should be treated differently from other bulky aggressive lymphomas arising at other anatomic sites.
原发性心脏淋巴瘤（PCL）在免疫功能正常的患者中极为罕见。已发表的系列研究采用了不同的定义标准。由于诊断延迟和疾病部位的相关性，预后较差。
报告了在研究机构观察到的2例病例，并回顾了1980年至1996年发表在文献中的48例病例。仅将淋巴瘤局限于心脏和/或心包以及仅有一个无症状心外部位的患者纳入分析。
8例患者有轻微的心外疾病。最常见的表现是难治性心力衰竭。心电图表现不具特异性。PCL通常起源于右心室，表现为肿块，伴或不伴有心包积液（>80%）。胸部X线、经胸超声心动图和计算机断层扫描是诊断检查的标准方法，但经食管超声心动图（TEE）和磁共振成像（MRI）的敏感性>90%。心包积液细胞学检查在67%的病例中具有诊断价值。开胸手术在所有病例中都具有诊断意义，而侵入性较小的检查有较高的假阴性率。根治性切除术无作用。早期含蒽环类药物的化疗似乎可提高生存率，而放疗的作用尚未明确。
对于有心脏肿块和/或不明原因难治性心包积液的患者应考虑PCL的诊断。充分的诊断检查，包括TEE和MRI，有助于证实对PCL的早期怀疑。在没有诊断性细胞学检查的情况下，可能需要进行开放性活检以避免治疗延迟。没有证据表明PCL的治疗应与其他解剖部位发生的其他大块侵袭性淋巴瘤有所不同。
