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病例报告:血栓栓塞与出血性心包积液——原发性心包血管肉瘤的双面性

Case Report: Thromboembolism and Hemorrhagic Pericardial Effusion-The Janus Face of Primary Pericardial Angiosarcoma.

作者信息

Chen Fei F, Jiang Shu F, Dong Chang, Che Ying, Du Lin Y, Li Zhi Y, Yang Zhi Q, Zhao Yi C, Liu Ying

机构信息

Department of Cardiology, The First Affiliated Hospital of Dalian Medical University, Dalian, China.

Department of Respiratory, The First Affiliated Hospital of Dalian Medical University, Dalian, China.

出版信息

Front Cardiovasc Med. 2021 Jan 15;7:618146. doi: 10.3389/fcvm.2020.618146. eCollection 2020.

Abstract

Primary cardiac angiosarcomas, especially those originating in the pericardium, are extremely rare and aggressive tumors with poor prognosis. These types of malignant tumors have diverse clinical presentations and are often masked by other comorbidities. Our hospital reported a 59-year-old woman who initially presented with pulmonary thromboembolism (PTE) and was subsequently treated with low-molecular-weight heparin. However, she experienced acute pericardial tamponade after anticoagulation therapy, where no obvious mass was primarily identified upon imaging, both in the pericardium or within the heart. Emergency pericardiocentesis and drainage were performed, where a total of 210 mL of bloody effusion was drained. Four months later, she was hospitalized with progressive hemoptysis and dyspnea. A large mixed mass occupying the right pericardium was later identified by coronary computed tomography angiography (CCTA). The mass was consistent with the right atrium, with heterogeneous thickened pericardium and localized moderate pericardial effusion. CCTA and positron emission tomography scans later showed metastases in both lungs and bilateral pleura. Nodules in hilar and mediastinal lymph nodes were also significant. Ultrasound-guided biopsy was performed, and the patient was ultimately diagnosed with an angiosarcoma based on final positive results for both CD31 and CD34 markers. The patient refused chemotherapy and passed away while waiting for her pathology results. The patient survived for 6 months since the first reported episode of PTE. Our case indicates that patients presenting with both embolism and hemorrhage should urgently be channeled to a clinical specialist to confirm any malignant etiology. This would be beneficial to confirm an early diagnosis and lengthen the duration of patient survival. However, the diagnosis of primary cardiac angiosarcoma is still challenging and requires multiple imaging modalities and biopsies in order to assist the accurate diagnosis of disease and achieve effective patient management.

摘要

原发性心脏血管肉瘤,尤其是起源于心包的血管肉瘤,是极其罕见且侵袭性强、预后差的肿瘤。这类恶性肿瘤临床表现多样,常被其他合并症掩盖。我院报告了一名59岁女性,最初表现为肺血栓栓塞症(PTE),随后接受低分子量肝素治疗。然而,抗凝治疗后她出现了急性心包填塞,影像学检查最初在心脏或心包内均未发现明显肿块。进行了紧急心包穿刺引流,共引出210毫升血性积液。四个月后,她因进行性咯血和呼吸困难住院。后来冠状动脉计算机断层扫描血管造影(CCTA)发现一个巨大的混合性肿块占据右心包。该肿块与右心房相连,心包不均匀增厚并伴有局限性中等量心包积液。CCTA和正电子发射断层扫描后来显示双肺和双侧胸膜有转移。肺门和纵隔淋巴结也有明显结节。进行了超声引导下活检,最终根据CD31和CD34标记物的最终阳性结果确诊为血管肉瘤。患者拒绝化疗,在等待病理结果时去世。自首次报告PTE发作以来,患者存活了6个月。我们的病例表明,出现栓塞和出血症状的患者应紧急转诊给临床专家以确认任何恶性病因。这将有助于早期诊断并延长患者生存期。然而,原发性心脏血管肉瘤的诊断仍然具有挑战性,需要多种影像学检查和活检来辅助准确诊断疾病并实现有效的患者管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0506/7843435/45fec60c87be/fcvm-07-618146-g0001.jpg

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