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原发性心脏血管肉瘤:一种罕见且致命的诊断。

Primary Cardiac Angiosarcoma: A Rare and Fatal Diagnosis.

作者信息

Gonçalves Inês, Nunes Catarina, Vieira Catarina, Freitas Diana, Pinto Luisa

机构信息

Internal Medicine, Hospital de Braga, Braga, PRT.

Cardiology, Hospital de Braga, Braga, PRT.

出版信息

Cureus. 2021 Dec 29;13(12):e20816. doi: 10.7759/cureus.20816. eCollection 2021 Dec.

Abstract

Primary cardiac angiosarcoma is a rare malignant neoplasm and occurs most frequently in middle-aged males. It has an aggressive nature, with highly variable clinical features, which results in delayed diagnosis and high mortality. We report a 19-year-old man presented to the ED with a three-month history of hemoptysis and one-week history of anterior chest pain. Additionally, an aortic diastolic murmur grade II/VI was found on physical examination. Thoracic CT scan revealed bilateral dispersed hypodense pulmonary nodes with peripheral halo, alveolar densification, and pericardial effusion. The transthoracic echocardiogram confirmed sizeable pericardial effusion and bicuspid aortic valve, without other significant findings. A pericardiocentesis removed 1300 mL of hemorrhagic fluid, consistent with an exudate without malignant cells. Both cardiac magnetic resonance and transesophageal echocardiogram revealed a large mass on the right atrium's anterior wall. Mass biopsy was performed, revealing malignant cardiac angiosarcoma. The biopsy of the lung lesions was compatible with lung metastasis of primary cardiac angiosarcoma. The patient was submitted to palliative chemotherapy but died 12 months after the diagnosis.

摘要

原发性心脏血管肉瘤是一种罕见的恶性肿瘤,最常发生于中年男性。它具有侵袭性,临床特征高度可变,导致诊断延迟和高死亡率。我们报告一名19岁男性因咯血3个月和前胸痛1周就诊于急诊科。此外,体格检查发现主动脉舒张期杂音二级/六级。胸部CT扫描显示双侧散在的低密度肺结节,伴有周边晕征、肺泡实变和心包积液。经胸超声心动图证实有大量心包积液和二叶式主动脉瓣,无其他显著发现。心包穿刺抽出1300毫升血性液体,符合渗出液,未发现恶性细胞。心脏磁共振成像和经食管超声心动图均显示右心房前壁有一个大肿块。进行了肿块活检,结果显示为恶性心脏血管肉瘤。肺部病变活检与原发性心脏血管肉瘤的肺转移相符。该患者接受了姑息化疗,但在诊断后12个月死亡。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa3a/8794415/5a8d535162fa/cureus-0013-00000020816-i01.jpg

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