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*Corresponding author: E-mail: tcvmisa@jichi.ac.jp;
www.sciencedomain.org
Cardiac Angiosarcoma of the Right Atrium with
Cardiac Tamponade
Hirotaka Sato
1, Kei Aizawa
1, Arata Muraoka
1, Hirohiko Akutsu
1and Yoshio Misawa
1*1
Division of Cardiovascular Surgery, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi, 329-0498, Japan.
Authors’ contributions
This work was carried out in collaboration between all authors. Author HS designed the study, wrote the protocol, and wrote the first draft of the manuscript. Author YM managed the literature searches and manuscript editing. Authors KA, AM and HA did the manuscript review. All authors read and approved the final manuscript.
Article Information
DOI: 10.9734/BJMMR/2016/22051 Editor(s): (1) Vijayalakshmi I. Balekundri, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bengaluru, India. Reviewers: (1) Zhen Yuan, University of Macau, China. (2)Zaffran Stéphane, Aix Marseille University, France. (3)Silvia Di Legge, University of Rome “Tor Vergata”, Italy. Complete Peer review History:http://sciencedomain.org/review-history/12179
Received 15th September 2015 Accepted 23rd October 2015 Published 9th November 2015
ABSTRACT
Aims: We report a case of cardiac sarcoma, which showed cardiac tamponade as the first clinical
symptom and sign. Accumulation of such cases could lead to better treatment for patients with cardiac angiosarcoma.
Presentation of Case: A 56-year-old man presented with loss of consciousness. An
echocardiogram showed cardiac tamponade, requiring an emergent exploration of the pericardium. We confirmed that the tumor originated in the right atrium and advanced to the right ventricle beyond the right coronary artery. A thickened right atrium with a small hole was resected, followed by reconstruction of the right atrium using a large patch of autologous pericardium. Cardiac angiosarcoma was pathologically identified. The patient died of multiple lung metastases 6 months after the operation, despite subsequent radiation therapy.
Discussion and Conclusion: Cardiac angiosarcoma shows extremely aggressive growth, as
observed in our case. More clinical reports with cardiac angiosarcoma could aid in improving diagnostic and treatment modalities.
Keywords: Cardiac tumor; angiosarcoma; cardiac tamponade; positron emission computed tomography.
ABBREVIATION
CT: Computed tomography
1. INTRODUCTION
Cardiac angiosarcoma,is the most common primary malignant tumor of the heart. Cardiac sarcoma can originate from the mesenchymal tissue or vascular endothelium, and it shows an extremely aggressive growth. The diagnosis of cardiac angiosarcoma is often delayed because of a death of symptoms and signs. Clinical evidence of pericardial effusion may help confirm the diagnosis of cardiac angiosarcoma. However, pericardial effusion tends to occur in advanced cases of cardiac angiosarcoma [1-3].We report a case of more advanced cardiac tamponade with cardiac angiosarcoma. More clinical reports with cardiac angiosarcoma could aid in improving diagnostic and treatment modalities.
2. PRESENTATION OF CASE
A 56-year-old man presented with loss of consciousness. Initial treatment included intra-tracheal intubation followed by mechanical ventilation for deteriorated hemodynamics. Subsequent investigations showed advanced cardiac tamponade requiring percutaneous pericardial drainage. The patient was referred to our hospital because of massive bloody effusion and active bleeding.
On examination, his blood pressure was 116/80 mmHg, and his heart rate was 100 bpm without arrhythmia. Normal heart sounds and normal vesicular sounds were audible. Laboratory findings showed a white blood cell count of 1,100/µl and a hemoglobin level of 9.8 g/dl. There was a slight elevation in levels of serum aspartate aminotransferase (46 mU/ml), alanine transaminase (72 mU/ml,) blood urea nitrogen (27 mg/dl), and creatinine (1.46 mg/dl).
A transthoracic echocardiographic examination showed mild pericardial effusion with an ejection fraction of 60% under pericardial drainage by a catheter. Chest computed tomography (CT) did not show acute aortic dissection or abnormal masses in the mediastinum and bilateral lung fields. There was persistent active drainage of blood, which required an emergent operation to control the bleeding.
Through standard median sternotomy and pericardiotomy, we found that the right atrium was covered with blood coagula. Removal of the coagula caused active bleeding from the right atrium. After establishment of a standard cardiopulmonary bypass, a hole with a diameter of 8 mm was observed under the coagula on the right atrial free wall (Fig. 1-a).
The hole was located on a thick wall of the atrium. We closed the hole by suture. The free wall surface along the right atrio-ventricular groove was rough, and the wall was thickened. We confirmed that the tumor originated in the right atrium and had advanced to the right ventricle beyond the right coronary artery. We resected the thickened right atrium including the closed hole, followed by reconstruction of the right atrium using a large patch of an autologous pericardium (Fig. 1-b). The total cardiopulmonary bypass time was 90 minutes.
The histological characteristics of the resected specimen were compatible with myocardial angiosarcoma, which showed abundant cellular proliferation (Fig. 2-a). Furthermore, an anastomosing vascular channel was present and had been formed by atypical endothelial cells (Fig. 2-b). Spindle cells and vascular walls showed intense immunostaining for CD31 (Fig. 2-c), CD34, vimentin, and factor VIII, and negative immunostaining for calponin, S100, KL1, and D2-40.
Postoperative echocardiography 2 weeks after the operation showed normal cardiac function without pericardial effusion and tricuspid valve regurgitation. Positron emission tomography CT showed a residual lesion of the right atrium (Fig. 3). The patient was discharged on the 30th day in hospital. Radiation therapy of 10 Gy per week was started for 6 weeks. After radiation therapy, the patient had a pneumothorax caused by multiple lung metastases. He then underwent pleurodesis and thoracoscopic pulmonary segmental resection to control repeated pneumothoraces. However, air leakage and active bleeding from the lungs could not be controlled. He finally died of multiple lung metastases 6 months after the initial operation.
3. DISCUSSION
series [1]. A total of 25% of primary cardiac tumors in adults are malignant, and 80% of malignant cardiac tumors are angiosarcoma [2]. Cardiac angiosarcomas occur more frequently in the right atrium, are more common in men, and have a tendency to occur in the 3rd and 5th decades of life [2].
Cardiac angiosarcomas do not typically present with significant clinical manifestations, and this may account for delayed diagnosis [3]. Unfortunately, cardiac angiosarcoma is often overlooked as an initial diagnosis because of its rarity. Therefore, preoperative diagnosis of catdiac angiosarcoma is almost impossible. Clinical manifestation of cardiac angiosarcoma includes dyspnea and palpitation caused by pericardial effusion. A definite diagnosis of cardiac angiosarcoma is obtained after operations, (e.g., for cardiac tamponade). Most cases of cardiac angiosarcoma are diagnosed incidentally or during screening examinations after pericardial effusion [4]. In case reports, cardiac angiosaromas are shown as a mass of the right atrium in CT scans and echocardiograms. Acute aortic dissection and acute myocardial infarction might cause cardiac tamponade, but they have characteristic CT or electrocardiographic findings.
In our case, we could not find a mass in the preoperative CT scans, and transthoracic echocardiograms. The patient’s hemodynamic status deteriorated with active bleeding. Therefore, we could not attempt other examinations in detail. We decided to stop the bleeding by surgery as soon as possible.
Surgical resection is considered the best choice of therapy for cardiac angiosarcoma, but the overall median survival is between 6 months and several years [4,5]. Complete resection of the tumor is difficult. Li and colleagues reported five cases with microscopically negative margin resection of cardiac sarcomas including angiosarcomas, and showed a median survival of 72 months [6]. Radiotherapy and chemotherapy for cardiac angiosarcomas are not well-established treatment modalities, and effects on extension of life are limited [5,7,8]. There are no prospective studies on angiosarcomas. Delayed diagnosis and rapid progress of cardiac angiosarcomas prevent its adequate treatment modalities from being improved. Cardiac transplantation might be an alternative treatment in case with no metastatic lesions. However, rapidly progressing metastatic lesions in the bilateral lung field made us abandon the option. A study of genotypes and prognoses of cardiac angiosarcoma has been under investigation.
1-a 1-b
Fig. 1. Operative findings
1a. A hole with a diameter of 8 mm was observed under coagula on the right atrial free wall. The hole is located on a thick wall of the atrium. The right atrial wall, including the hole, is partially thickened (wide arrows) 1b. We resected the thickened right atrium, including the hole, followed by reconstruction of the right atrium,
2-a 2-b 2-c
Fig. 2. Histological findings
Fig. 2a. Abundant cellular proliferation is shown; Fig. 2b. An anastomosing vascular channel is formed by atypical endothelial cells; Fig. 2c. Spindle cells and vascular walls show intense immunostaining for CD31
Fig. 3. Postoperative positron emission computed tomography
The residual lesion is shown as a red area in the right atrium
Hydro-pneumothorax is sometimes associated with lung metastasis of angiosarcoma [9]. This condition develops from spontaneous rupture of cystic lesions of metastatic angiosarcomas. Our patient had a residual tumor as shown by positron emission tomography CT [10]. He also developed repeated pneumothorax, and pleurodesis and segmental resections were not effective, causing lethal results.
Histological findings of angiosarcomas are characteristic. Atypical cells have short spindle area and are round. The nucleus is vesicular with frequent mitotic Figures, and tumor necrosis is common. These atypical cells are immuno-histochemically positive for CD31, CD34, vimentin, and focal factor V . However, atypical cells are negative for calponin, S100, KL1, D2-40, and human herpesvirus 8 [11,12]. These immunological findings are characteristic of angiosarcomas.
4. CONCLUSIONS
We experienced a case of cardiac angiosarcoma with hemodynamic deterioration caused by cardiac tamponade. Resection of the tumor of the right atrium resulted in a limited 6 months after the operation despite radiation therapy. We may be able to obtain better diagnostic and treatment modalities in the future based on more clinical reports on cardiac angiosarcoma.
CONSENT
All authors declare that written informed consent in Japanese was obtained from patient’s wife for publication of this case report and any accompanying images.
ETHICAL APPROVAL
It is not applicable.
COMPETING INTERESTS
Authors have declared that no competing interests exist.
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© 2016 Sato et al.; This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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