WCC 2016-115: Cardiac Angiosarcoma of the Right Atrium: Two Cases

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.

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Cardiac Angiosarcoma on the Right Atrium: Two Cases

The Korean Journal of Thoracic and Cardiovascular Surgery ◽

10.5090/kjtcs.2012.45.2.120 ◽

2012 ◽

Vol 45 (2) ◽

pp. 120-123 ◽

Cited By ~ 6

Author(s):

Won Kyoun Park ◽

Sung-Ho Jung ◽

Ju Yong Lim

Keyword(s):

Right Atrium ◽

Cardiac Angiosarcoma ◽

The Right

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Cardiac Angiosarcoma of the Right Atrium with Cardiac Tamponade

British Journal of Medicine and Medical Research ◽

10.9734/bjmmr/2016/22051 ◽

2016 ◽

Vol 12 (5) ◽

pp. 1-5

Author(s):

Hirotaka Sato ◽

Kei Aizawa ◽

Arata Muraoka ◽

Hirohiko Akutsu ◽

Yoshio Misawa

Keyword(s):

Cardiac Tamponade ◽

Right Atrium ◽

Cardiac Angiosarcoma ◽

The Right

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Rapidly progressive respiratory failure with multiple halo signs on computed tomography in a patient with primary cardiac angiosarcoma derived from the right atrium: a case report

BMC Pulmonary Medicine ◽

10.1186/s12890-020-01366-6 ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

Masaoki Saito ◽

Takeshi Saraya ◽

Miku Oda ◽

Toshinori Minamishima ◽

Ken Kongoji ◽

...

Keyword(s):

Computed Tomography ◽

Cardiac Output ◽

Respiratory Failure ◽

Lung Tissue ◽

Right Atrium ◽

Cardiac Ct ◽

Loss Of Consciousness ◽

Cardiac Angiosarcoma ◽

Thoracic Ct ◽

The Right

Abstract Background Primary cardiac neoplasms are extremely rare, with an autopsy incidence of 0.0001–0.003%. Primary cardiac sarcoma is usually derived from the right atrium and it manifests as chest pain, arrhythmia, hemoptysis, dyspnea, and fatigue. The most common target organ for metastasis of primary angiosarcoma is the lungs, but the radiological-pathological correlation has been rarely reported. Case presentation A 38-year-old healthy Japanese man was admitted to our hospital with persistent hemoptysis, exaggerated dyspnea, and two episodes of loss of consciousness in the past 3 months. Non-enhanced thoracic computed tomography (CT) revealed multiple scattered nodules with halo signs. Contrast-enhanced thoracic CT revealed a filling defect in the right atrium, which corresponded to the inhomogeneously enhancing tumor in the right atrium on enhanced electrocardiogram-gated cardiac CT. On day 2, acute respiratory failure occurred, and the patient was placed on mechanical ventilation. The patient was diagnosed with primary cardiac angiosarcoma based on the urgent transcatheter biopsied specimen of the right atrium mass and was treated with intravenous administration of doxorubicin. However, his respiratory status rapidly deteriorated, and he died on day 20. Postmortem biopsy showed that the multiple lung nodules with the halo signs corresponded to the intratumoral hemorrhagic necrosis and peripheral parenchymal hemorrhage in their background, suggesting the fragility of the lung tissue where the tumor had invaded, which caused hemoptysis. Furthermore, two episodes of loss of consciousness occurred probably due to a decreased cardiac output because of a massive tumor occupying the right atrium, recognized as an inhomogeneous centripetal enhancement on enhanced electrocardiogram-gated cardiac CT. Conclusions This case clearly demonstrated that primary cardiac angiosarcoma could expand in the right atrial cavity, which led to a decreased cardiac output resulting in repeated syncope, together with the fragility of lung tissue by tumor invasion, thereby generating a halo sign on thoracic CT.

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Surgical Treatment of a Giant Primary Cardiac Angiosarcoma

Acta Medica (Hradec Kralove Czech Republic) ◽

10.14712/18059694.2017.31 ◽

2008 ◽

Vol 51 (4) ◽

pp. 237-239 ◽

Cited By ~ 1

Author(s):

Maruf Sanli ◽

Bulent Tuncozgur ◽

Alper Sevinc ◽

Bahadir Daglar ◽

Kemal Bakir ◽

...

Keyword(s):

Right Ventricle ◽

Surgical Resection ◽

Right Atrium ◽

Combined Treatment ◽

Histopathological Diagnosis ◽

Cardiac Angiosarcoma ◽

Resected Tumor ◽

Well Differentiated ◽

The Right ◽

Large Primary

Primary cardiac angiosarcoma is a rare tumor, and surgical resection is often required to relieve its symptoms. A 54-year-old male with a large primary cardiac angiosarcoma is described in this case report. The tumor was located in the right atrium and right ventricle. The bulk was resected with the right coronary artery (RCA), and partial right atrium and partial right ventricle resections were performed during cardiopulmonary bypass. The resected tumor measured 15 x 10 x 8 cm, and the histopathological diagnosis was well differentiated primary cardiac angiosarcoma. In the postoperative period, the patient was followed up for 22 months, and radiotherapy and chemotherapy were performed for metastases. The optimal therapy for cardiac angiosarcoma is still controversial, but combined treatment including surgical resection should be considered.

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Cardiac Angiosarcoma

Case Reports in Cardiology ◽

10.1155/2011/340681 ◽

2011 ◽

Vol 2011 ◽

pp. 1-3

Author(s):

Monique Esteves Cardoso ◽

Leonardo Secchin Canale ◽

Rosana Grandelle Ramos ◽

Edson da Silva Salvador Junior ◽

Stephan Lachtermacher

Keyword(s):

Adjuvant Therapy ◽

Right Atrium ◽

Malignant Tumors ◽

Tumor Resection ◽

Cardiac Tumors ◽

Primary Tumors ◽

Cardiac Angiosarcoma ◽

Cardiac Metastases ◽

The Right ◽

Primary Cardiac Tumors

Despite cardiac metastases are found in about 20% of cancer deaths, the presence of primary cardiac tumors is rare. Most primary tumors are benign, and malignant tumors comprise about 15%. We report a 21-year-old man with fever, dyspnea, and hemoptysis that was diagnosed with angiosarcoma of the right atrium and pulmonary metastasis. Patient was submitted to surgical tumor resection without adjuvant therapy and died four months after diagnosis.

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Angiosarcoma of the Right Atrium with Extension to SVC and IVC Presenting with Complete Heart Block and Significant Pericardial Effusion

Case Reports in Cardiology ◽

10.1155/2016/3173069 ◽

2016 ◽

Vol 2016 ◽

pp. 1-3

Author(s):

Hossein Vakili ◽

Isa Khaheshi ◽

Mehdi Memaryan ◽

Shooka Esmaeeli

Keyword(s):

Pericardial Effusion ◽

Right Atrium ◽

Heart Block ◽

Complete Heart Block ◽

Histological Subtype ◽

Preliminary Diagnosis ◽

Cardiac Neoplasms ◽

Cardiac Angiosarcoma ◽

Healthy Man ◽

The Right

Primary cardiac neoplasms are particularly unusual. Angiosarcoma is the most frequently seen histological subtype and is described by its infiltrating and damaging nature. Inappropriately, primary cardiac angiosarcoma is often missed as a preliminary diagnosis because of its scarcity. We present a 29-year-old previously healthy man with complete heart block and pericardial effusion who was finally diagnosed with angiosarcoma of the right atrium with extension to SVC and IVC.

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Primary Cardiac Angiosarcoma of the Right Atrium Undiagnosed by Transvenous Endocardial Tumor Biopsy.

Internal Medicine ◽

10.2169/internalmedicine.37.1023 ◽

1998 ◽

Vol 37 (12) ◽

pp. 1023-1026 ◽

Cited By ~ 10

Author(s):

Ryo NITTA ◽

Yasunari SAKOMURA ◽

Kyoumi TANIMOTO ◽

Tomoaki HIDAI ◽

Hiroshi KASANUKI ◽

...

Keyword(s):

Right Atrium ◽

Tumor Biopsy ◽

Cardiac Angiosarcoma ◽

The Right

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DEATH DUE TO CARDIAC ANGIOSARCOMA: AUTOPSY CASE REPORT

International Journal of Medicine and Medical Research ◽

10.11603/ijmmr.2413-6077.2019.1.10155 ◽

2019 ◽

Vol 5 (1) ◽

pp. 17-20

Author(s):

F. Eren ◽

N. T. Inanır ◽

M. S. Gurses ◽

B. Eren ◽

U. N. Gundogmus ◽

...

Keyword(s):

Right Atrium ◽

Emergency Service ◽

Autopsy Case ◽

Primary Tumors ◽

Cardiac Angiosarcoma ◽

Legal Literature ◽

Cardiology Clinic ◽

Nodular Lesions ◽

The Right ◽

Histopathologic Findings

Background. Primary tumors of the heart are rarely detected at autopsy, especially angiosarcomas which are primary malignant one. Objective. We presented autopsy case of cardic angiosarcoma with morphologic findings. Methods. We described adult man died in emergency service of the hospital. Results. Reported case was 33 year-old-man who was died in emergency service of hospital where he was taken when he was ill after leaving home. According the prosecution documents, and the expressions of family, it was reported that he had a heart disease; his symptoms repeated 3 day ago before he died, he thought to attend the Cardiology Clinic due to his symptoms. At autopsy on macroscopic internal examination, mass with rough surface in the right atrium, hematoma at the posterior of the right atrium, blood in the pericardia, nodular lesions in hemorrhagic appearance in the sections of lung, liver and spleen were detected. In histopathologic examination; in the heart angiosarcioma as primary malign heart tumor and metastatic masses in the liver, spleen and lung were detected. Conclusions. We aimed to discuss cardiac angiosarcoma case with autopsy and histopathologic findings in the aspect of medico legal literature.

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Primary Cardiac Angiosarcoma: A Fatal Disease

Case Reports in Medicine ◽

10.1155/2009/591512 ◽

2009 ◽

Vol 2009 ◽

pp. 1-4 ◽

Cited By ~ 15

Author(s):

L. Antonuzzo ◽

V. Rotella ◽

F. Mazzoni ◽

L. Doni ◽

D. Bianchini ◽

...

Keyword(s):

Cardiac Tamponade ◽

Cardiac Mri ◽

Right Atrium ◽

Partial Remission ◽

Fatal Disease ◽

Cardiac Angiosarcoma ◽

Progression Of Disease ◽

The Right ◽

Oncology Unit ◽

Work Up

A 42-year-old man with a cardiac tamponade underwent an urgent pericardiotomy that showed tumoral tissue, covering the surface of the right atrium. The tumor was then partially excised, and the histological examination revealed the presence of a moderately-differentiated angiosarcoma. The patient was then referred to the oncology unit and scheduled for a chemotherapy schedule including Epirubicin (60 mg/m2, on days 1 and 2) plus Ifosfamide (2000 mg/m2, on days 1 to 3) and Uromitexan (2000 mg/m2at hours 0, 4, 8 after IFO). All drugs were administered every three weeks. After two cycles, a restaging work-up revealed a partial remission. The treatment was continued for another two cycles. A new evaluation by cardiac MRI evidenced a local and distant (lung) progression of disease. The patient died after three months. This paper confirms that cardiac angiosarcoma is a fatal disease, and the prognosis is usually 6–11 months from time of diagnosis.

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