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弥漫性肺泡出血作为抗磷脂综合征孕妇心脏血管肉瘤的临床表现:病例报告及文献复习

Diffuse alveolar hemorrhage as a clinical manifestation of cardiac angiosarcoma in a pregnant woman with antiphospholipid syndrome: case report and review of the literature.

作者信息

Hermosilla R, Martínez S, Perez A, López E, Granell R

机构信息

Department of Gynecology and Obstetrics, Hospital Juan Ramón Jiménez, Huelva 21005, Spain.

Department of Anatomic Pathology, Hospital Juan Ramón Jiménez, Huelva 21005, Spain.

出版信息

Ecancermedicalscience. 2025 Apr 1;19:1881. doi: 10.3332/ecancer.2025.1881. eCollection 2025.

Abstract

Primary cardiac angiosarcoma is a very rare and fast-growing tumour, where the coincidence of pregnancy and primary cardiac angiosarcoma is extremely rare. This makes diagnosis difficult and sometimes late, resulting in a poor prognosis from the moment of detection. We present the case of a 38-year-old pregnant woman in the 16th week of gestation diagnosed with obstetric antiphospholipid syndrome who came to the emergency department with asthenia, dyspnea, tachycardia and hypotension. A transthoracic ultrasound was performed with a diagnosis of pericardial tamponade. She was admitted to the intensive care unit for extrinsic cardiogenic shock. Transesophageal echography was performed, showing a large variegated mass in the right atrium, raising the differential diagnosis between atrial thrombus and myocardial tumour. Elective cesarean section was performed at 22 weeks of gestation. Months later, the patient was readmitted with a very unfavorable clinical evolution, experiencing diffuse alveolar hemorrhage/vasculitis refractory to life support measures. It was agreed to limit the therapeutic effort while awaiting the anatomopathological report of intracardiac biposia, subsequently confirming the histological nature of cardiac angiosarcoma. This case report highlights its rarity, showing a non-specific clinical presentation, which directed us towards a thrombosis in the right atrium and the fatal prognosis of angiosarcoma, mainly related to the progression of the disease due to a late diagnosis.

摘要

原发性心脏血管肉瘤是一种非常罕见且生长迅速的肿瘤,而妊娠与原发性心脏血管肉瘤同时存在的情况极为罕见。这使得诊断困难,有时甚至延迟,导致从发现之时起预后就很差。我们报告一例38岁妊娠16周的孕妇,诊断为产科抗磷脂综合征,因乏力、呼吸困难、心动过速和低血压前来急诊科就诊。进行了经胸超声检查,诊断为心包填塞。她因心外源性休克入住重症监护病房。进行了经食管超声心动图检查,显示右心房有一个大的杂色肿块,这增加了心房血栓和心肌肿瘤之间的鉴别诊断难度。在妊娠22周时进行了择期剖宫产。数月后,患者因临床病情进展极为不利再次入院,出现弥漫性肺泡出血/血管炎,对生命支持措施无效。在等待心内活检的解剖病理学报告期间,同意限制治疗措施,随后报告证实了心脏血管肉瘤的组织学性质。本病例报告突出了其罕见性,显示出非特异性的临床表现,这使我们最初考虑为右心房血栓形成,以及血管肉瘤的致命预后,主要与诊断延迟导致的疾病进展有关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8e5b/12149227/e214c0942f8a/can-19-1881fig1.jpg

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