罕见的右心房未分化多形性肉瘤合并中央坏死1例。

Zorica Mladenovic, Slobodan Obradovic, Sasa Ristic, Ljubinko Djenic, Edin Begic
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引用次数: 0

摘要

原发性心脏肿瘤是一种罕见的肿瘤类型,根据其位置的不同,可以表现出多种临床体征和症状。我们提出一个病例的57岁的女性患者与严重的右侧心力衰竭。通过二维经胸和三维经食管超声心动图检查,发现右心房内有一个巨大的椭圆形肿瘤样物,妨碍了上下腔静脉的血液流动。它的中心部分似乎有一个不规则的无回声空间,可能是由于坏死。胸部多层计算机断层扫描显示右心房一异质、膨胀性肿瘤样肿块,中心有出血征象。虽然没有转移扩散的迹象,但不能排除肿瘤样肿块起源于心脏外。病人接受了手术切除肿瘤。手术伴有出血并发症,这是由于中央坏死和局部浸润引起的。术后出现严重的全身炎症反应综合征,患者死亡。病理诊断为未分化多形性心脏肉瘤,其预后通常较差。这种诊断的患者的中位生存期不到1年,即使手术切除和进一步的辅助治疗。
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A Rare Case of Undifferentiated Pleomorphic Sarcoma with Central Necrosis in the Right Atrium.

Primary cardiac tumors, which are uncommon types of tumors, can be presented with a variety of clinical signs and symptoms, depending on their location. We present a case of a 57-year-old female patient with a severe right-sided heart failure. Examination using 2-dimensional transthoracic and 3-dimensional transoesophageal echocardiography detected a large, oval, tumor-like formation within the right atrium, which compromised the blood flow from the superior and inferior vena cava. It appeared to have an irregular echo-free space in its central part, probably due to necrosis. Thoracic multislice computed tomography revealed a heterogeneous, expansive, tumor-like mass in the right atrium, with signs of bleeding in its center. Although there were no signs of metastatic dissemination, it could not be excluded that the tumor-like mass originated outside of the heart. The patient underwent surgical resection of the tumor. The surgery was accompanied with bleeding complications that developed due to the central necrosis with local infiltration. During the postoperative period, severe systemic inflammatory response syndrome developed and the patient died. Pathologists diagnosed undifferentiated pleomorphic cardiac sarcoma for which the prognosis is usually poor. The median survival of patients with this type of diagnosis is less than 1 year, even with surgical resection and further adjuvant therapy.

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来源期刊
CiteScore
1.30
自引率
12.50%
发文量
124
审稿时长
32 weeks
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