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心脏血管肉瘤:病例报告。

Cardiac angiosarcoma: a case report.

机构信息

Assistant Professor of Cardiac Surgery, Department of Cardiovascular Surgery, Modarres Hospital, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Researcher, Department of Cardiovascular Surgery, Modarres Hospital, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

出版信息

J Int Med Res. 2023 Nov;51(11):3000605231211772. doi: 10.1177/03000605231211772.

Abstract

Angiosarcoma is the most invasive and malignant cardiac tumor and most commonly originates from the right atrium. Early diagnosis is essential, and echocardiography has an important role in diagnosis. This tumor grows aggressively, and metastases to other sites makes it difficult to control. Surgical treatment remains the best option for patients who do not respond to chemoradiotherapy. We herein report a case of a 17-year-old patient with cardiac angiosarcoma who presented with dyspnea, chest pain, dry cough, and fever. Although we considered the most probable diagnosis to be constrictive pericarditis, pathologic examination revealed a primary angiosarcoma originating from the pericardium. The patient underwent total pericardiectomy. However, despite receiving chemotherapy for 2 weeks postoperatively, she developed complications including leukopenia and eventually died of respiratory failure. Late diagnosis of angiosarcoma often occurs, resulting in progression to end-stage disease and a very poor prognosis. Therefore, a thorough understanding of this entity, knowledge of its pitfalls in management, and establishment of an accurate treatment guideline would help to develop a reliable and life-saving treatment approach for these patients.

摘要

血管肉瘤是最具侵袭性和恶性的心脏肿瘤,最常起源于右心房。早期诊断至关重要,超声心动图在诊断中具有重要作用。这种肿瘤生长迅速,转移到其他部位使其难以控制。对于对放化疗无反应的患者,手术治疗仍是最佳选择。我们在此报告一例 17 岁的心脏血管肉瘤患者,表现为呼吸困难、胸痛、干咳和发热。尽管我们考虑最可能的诊断为缩窄性心包炎,但病理检查显示原发性心包血管肉瘤起源。患者接受了全心包切除术。然而,尽管术后接受了 2 周的化疗,她还是出现了白细胞减少等并发症,最终因呼吸衰竭而死亡。血管肉瘤的晚期诊断较为常见,导致疾病进展至终末期,预后极差。因此,深入了解该疾病,了解其在治疗中的误区,并制定准确的治疗指南,将有助于为这些患者制定可靠的、救命的治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db73/10664435/85af0b4b3309/10.1177_03000605231211772-fig1.jpg

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