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原发性心脏血管肉瘤侵犯右房室交界和三尖瓣的切除术:一例报告。

Resection of primary cardiac angiosarcoma infiltrating the right atrioventricular junction and tricuspid valve: a case report.

机构信息

Department of Cardiac Surgery, Faculty of Medicine, Damascus University, Damascus, Syria.

出版信息

J Cardiothorac Surg. 2021 Mar 21;16(1):41. doi: 10.1186/s13019-021-01426-w.

Abstract

BACKGROUND

Primary cardiac tumours are extremely rare. Most of them are benign. Sarcomas account for 95% of the malignant tumours. Prognosis of primary cardiac angiosarcoma remains poor. Complete surgical resection is oftentimes hampered when there is extensive tumour involvement into important cardiac apparatus. We report a case of cardiac angiosarcoma of the right atrium and ventricle, infiltrating the right atrioventricular junction and tricuspid valve.

CASE PRESENTATION

Initially, a 22-year-old man presented with dyspnoea. One year later, he had recurrent pericardial effusion. Afterwards, echocardiography revealed a large mass in the right atrium, expanding from the roof of the right atrium to the tricuspid valve. The mass was causing compression on the tricuspid valve, and another mass was seen in the right ventricle. Complete resection of the tumour was impossible. The mass was resected with the biggest possible margins. The right atrium was reconstructed using heterologous pericardium. The patient's postoperative course was uneventful. Postoperative echocardiography showed a small mass remaining in the right side of the heart. Histopathology and immunohistochemistry confirmed the diagnosis of angiosarcoma. The patient underwent adjuvant chemotherapy and radiotherapy later on. He survived for 1 year and 5 days after the surgery. After a diagnosis of lung and brain metastases, he ended up on mechanical ventilation for 48 h and died.

CONCLUSIONS

Surgical resection combined with postoperative chemotherapy and radiotherapy is feasible even in patients with an advanced stage of cardiac angiosarcoma when it is impossible to perform complete surgical resection.

摘要

背景

原发性心脏肿瘤极为罕见,其中大多数为良性。肉瘤占恶性肿瘤的 95%。原发性心脏血管肉瘤的预后仍然较差。当肿瘤广泛侵犯重要的心脏器官时,常常难以进行完全的手术切除。我们报告了 1 例右心房和右心室的心脏血管肉瘤,浸润右房室结和三尖瓣的病例。

病例介绍

最初,一名 22 岁男性因呼吸困难就诊。1 年后,他出现复发性心包积液。随后,超声心动图显示右心房内有一个大肿块,从右心房顶部扩展到三尖瓣。该肿块压迫三尖瓣,右心室也有一个肿块。完全切除肿瘤是不可能的,因此我们尽可能地扩大切除肿瘤的范围。用同种异体心包重建右心房。患者术后情况平稳。术后超声心动图显示右心仍有一小肿块。组织病理学和免疫组化检查证实了血管肉瘤的诊断。随后患者接受了辅助化疗和放疗。他在手术后存活了 1 年零 5 天。在诊断为肺和脑转移后,他最终接受了 48 小时的机械通气,并死亡。

结论

即使在无法进行完全手术切除的情况下,对于晚期心脏血管肉瘤患者,手术切除联合术后化疗和放疗也是可行的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/49f2/7981869/a1f88f961e3e/13019_2021_1426_Fig1_HTML.jpg

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