Recurrent pericardial effusion from primary cardiac angiosarcoma leading to delayed diagnosis： a case report and literature review

doi:10.3969/j.issn.1009-976X.2023.04.009

Abstract

Abstract: Primary cardiac angiosarcoma is very rare disease with very poor prognosis due to its high mortality, late diagnosis, and lack of effective treatment. Early diagnosis may relieve patient′s symptoms and prolong survival. This article presented the case of a 38 year-old male patient with recurrent persistent pericardial effusion for three months as the first symptom from primary cardiac angiosarcoma which leading to delayed diagnosis and treatment. As soon as the clinical diagnosis of localized right atrial angiosarcoma without metastasis was made, the surgical resection was performed and the right atrium was reconstructed with a bovine pericardium patch. Postoperative pathological examination confirmed the preoperative clinical diagnosis being moderate differentiated primary right atrial angiosarcoma. The patient received chemotherapy with Duopafei （Docetaxel from Qilu Pharmaceutical Co., Ltd.） for three cycles. Lung metastasis and local recurrence were found 4 months after operation, and the patient died 3 months later. For patients with recurrent or unexplained pericardial effusion, echocardiography, cardiac CTA and MR imaging techniques are recommended for early detection and diagnosis of primary cardiac angiosarcoma.

Key words: cardiac angiosarcoma, surgical resection, chemotherapy, radiation therapy

摘要： 原发性心脏血管肉瘤是一种非常罕见的疾病,因其高死亡率、早期诊断困难和缺乏有效治疗,预后非常差。早期诊断可以缓解患者症状,延长生存时间。本文报道一位38岁男性,患者以持续性心包积液为首发症状持续三个月,最终被诊断为原发性心脏血管肉瘤。在临床诊断为无转移的右心房血管肉瘤后立即进行手术切除,并用牛心包补片重建右心房。术后病理证实了术前的临床诊断,为中度分化的原发性右心房血管肉瘤。术后患者接受了三个疗程的多西他赛化疗,术后4个月发现肺转移和局部复发,于术后7个月去世。对于有复发或原因不明的心包积液的患者,建议进行超声心动图、心脏CTA和MR等检查,以早期检测和诊断原发性心脏血管肉瘤。

关键词: 心脏血管肉瘤, 手术切除, 化疗, 放射治疗

CLC Number:

R654.2

LIU Cong-yong, ZHANG Lu, JIANG Hui-qi, GAO Min-nan, LI Jing-wen, SHI Zhan-yue, YANG Yan-qi, ZHENG Jun-meng. Recurrent pericardial effusion from primary cardiac angiosarcoma leading to delayed diagnosis： a case report and literature review[J]. Lingnan Modern Clinics In Surgery, 2023, 23(04): 337-342.

刘丛勇, 张露, 江慧琦, 高敏楠, 李婧雯, 石展岳, 杨艳旗, 郑俊猛. 原发性心脏血管肉瘤导致反复的心包积液：病例报告和文献回顾[J]. 岭南现代临床外科, 2023, 23(04): 337-342.

References

[1] Karki DS, Westhoff M, Maschek H, et al.A rare diagnostic challenge in a female patient with a rapid recurrent pleural effusion: Autopsy revealed cardiac angiosarcoma with bilateral pleural and pulmonary metastases. A case report[J]. Int J Surg Case Rep, 2021, 78: 278-283.
[2] Hamidi M, Moody JS, Weigel TL, et al.Primary Cardiac Sarcoma[J]. Ann Thorac Surg, 2010, 90(1): 176-181.
[3] Krishnan T, Pettersson G, Mukherjee R, et al.Cardiac angiosarcoma: A diagnostic and therapeutic challenge[J]. J Cardiol Cases, 2020, 22(2): 90-93.
[4] Loukas M.Primary cardiac angiosarcoma-a review[J]. Med Sci Monit, 2014, 20: 103-109.
[5] Mayer F, Aebert H, Rudert M, et al.Primary malignant sarcomas of the heart and great vessels in adult patients--a single-center experience[J]. Oncologist, 2007, 12(9): 1134-1142.
[6] Butany J, Yu W, et al.Cardiac angiosarcoma: two cases and a review of the literature[J]. Can J Cardiol, 2000, 16(2): 197.
[7] Chaves VM, Pereira C, Andrade M, et al.Cardiac Angiosarcoma: From Cardiac Tamponade to Ischaemic Stroke-A Diagnostic Challenge[J]. Eur J Case Rep Intern Med, 2019, 6(4): 1.
[8] Ge Y, Ro JY, Kim D, et al.Clinicopathologic and immunohistochemical characteristics of adult primary cardiac angiosarcomas: analysis of 10 cases[J]. Ann Diagn Pathol, 2011, 15(4): 262-267.
[9] Lin C, Ducis K, Tucker S, et al.Metastatic Cardiac Angiosarcoma to the Lung, Spine, and Brain: A Case Report and Review of the Literature[J]. World Neurosurg, 2017,107: 1049.
[10] Guo S, Guo Q.Primary Cardiac Angiosarcoma[J]. Int Heart J, 2021, 62(2): 458-462.
[11] Look HN, Pandalai PK, Hornick JL, et al.Cardiac angiosarcoma management and outcomes: 20-year single-institution experience[J]. Ann Surg Oncol, 2012, 19(8): 2707-2715.
[12] Ananthasubramaniam K, Farha A.Primary right atrial angiosarcoma mimicking acute pericarditis, pulmonary embolism, and tricuspid stenosis[J]. Heart, 1999, 81(5): 556-558.
[13] Krishnan T, Pettersson G, Mukherjee R, et al.Cardiac angiosarcoma: A diagnostic and therapeutic challenge[J]. J Cardiol Cases, 2020, 22(2): 90-93.
[14] Riles E, Gupta S, Wang DD, et al.Primary cardiac angiosarcoma: A diagnostic challenge in a young man with recurrent pericardial effusions[J]. Exp Clin Cardiol, 2012, 17(1): 39-42.
[15] Sultan I, Bianco V, Habertheuer A, et al.Long-Term Outcomes of Primary Cardiac Malignancies[J]. J Am Coll Cardiol, 2020, 75(18): 2338-2347.
[16] Ge Y, Ro JY, Kim D, et al.Clinicopathologic and immunohistochemical characteristics of adult primary cardiac angiosarcomas: analysis of 10 cases[J]. Ann Diagn Pathol, 2011, 15(4): 262-267.
[17] Antonuzzo L, Rotella V, Mazzoni F, et al.Primary Cardiac Angiosarcoma: A Fatal Disease[J]. Case Rep Med, 2009, 2009: 1-4.
[18] Blackmon SH, Reardon MJ.Surgical treatment of primary cardiac sarcomas[J]. Tex Heart Inst J, 2009, 36(5): 451-452.
[19] Sinatra R, Brancaccio G, di Gioia CRT, et al. Integrated approach for cardiac angiosarcoma[J]. Int J Cardiol, 2003, 88(2-3): 301-304.
[20] Simpson L, Kumar SK, Okuno SH, et al.Malignant primary cardiac tumors[J]. Cancer, 2008, 112(11): 2440-2446.
[21] Piazza N, Chughtai T, Toledano K, et al.Primary cardiac tumours: eighteen years of surgical experience on 21 patients[J]. Can J Cardiol, 2004, 20(14): 1443-1448.
[22] Nakamura-Horigome M, Koyama J, Eizawa T, et al.Successful Treatment of Primary Cardiac Angiosarcoma With Docetaxel and Radiotherapy[J]. Angiology, 2008, 59(3): 368-371.
[23] Jang Y, Kim J, Shim JW, et al.Primary cardiac angiosarcoma: a prolonged response to surgical resection followed by concurrent chemoradiotherapy with docetaxel[J]. Springer Plus, 2016, 5(1): 155.
[24] Urbini M, Astolfi A, Indio V, et al.Genetic aberrations and molecular biology of cardiac sarcoma[J]. Ther Adv Med Oncol, 2020, 12: 1-14.