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以心脏压塞为表现的右心房原发性心脏未分化肉瘤。

Primary cardiac undifferentiated sarcoma of the right atrium presenting with cardiac tamponade.

作者信息

Kong Poi Keong, Theodorou Stasinos, Beran Elisabeth, Singh Hardial, Dimitri Wadih

机构信息

Department of Cardiology, University Hospital, University Hospitals Coventry and Warwickshire NHS Trust, United Kingdom.

出版信息

Cardiovasc Pathol. 2009 Mar-Apr;18(2):110-3. doi: 10.1016/j.carpath.2007.10.006. Epub 2007 Dec 3.

Abstract

This report illustrates the serial imaging of a primary cardiac undifferentiated sarcoma of the right atrium using echocardiography, chest X-ray, and computed tomography. Transthoracic echocardiography on presentation showed an extensive mass of the right atrial free wall with an impending cardiac tamponade. Symptoms were controlled with pericardiocentesis, pericardial window, and radiotherapy but recurred 8 months later with pleural effusion and tumor spread to the great arteries. Primary cardiac sarcoma (PSC) is a rare and aggressive malignancy that is usually diagnosed late due to its nonspecific symptoms. Cytology and cardiac biopsy may be negative, and suspicion for the tumor is warranted in recurrent pericardial effusion. Analogous to parietal pleural biopsy in lung tumors with pleural effusion, parietal pericardial biopsy may be positive in PSC of the right atrium with pericardial effusion. Echocardiography is the major diagnostic tool and aids pericardiocentesis. Pericardial window may be useful for recurrent pericardial effusion but does not preclude its reaccumulation. There is no proven effective treatment for PSC, and treatments include surgical resection, cardiac transplant, chemotherapy, and radiotherapy. Despite its poor prognosis, symptomatic relief is important and attainable.

摘要

本报告展示了使用超声心动图、胸部X线和计算机断层扫描对一例右心房原发性心脏未分化肉瘤进行的系列成像。初诊时经胸超声心动图显示右心房游离壁有一巨大肿块,并有心脏压塞的危险。通过心包穿刺、心包开窗术和放射治疗控制了症状,但8个月后复发,出现胸腔积液且肿瘤扩散至大动脉。原发性心脏肉瘤(PSC)是一种罕见且侵袭性强的恶性肿瘤,由于其症状不具特异性,通常诊断较晚。细胞学检查和心脏活检可能为阴性,对于复发性心包积液,应怀疑有该肿瘤。类似于对有胸腔积液的肺肿瘤进行壁层胸膜活检,对有心包积液的右心房PSC进行壁层心包活检可能为阳性。超声心动图是主要的诊断工具,有助于心包穿刺。心包开窗术可能对复发性心包积液有用,但不能防止其再次积聚。目前尚无已证实对PSC有效的治疗方法,治疗包括手术切除、心脏移植、化疗和放疗。尽管预后较差,但缓解症状很重要且可以实现。

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