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      Successful surgical excision of primary right atrial angiosarcoma

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          Abstract

          Primary cardiac angiosarcoma is a rare and aggressive tumor with a high incidence of metastatic spread (up to 89%) at the time of diagnosis, which restricts the indication for surgical resection to a small number of patients. We report the case of a 50-year old Caucasian woman with non-metastatic primary right atrial angiosarcoma, who underwent successful surgical excision of the tumor (with curative intent) and reconstruction of the right atrium with a porcine pericardial patch. However, after a symptom-free survival of five months the patient presented with bone and liver metastases without evidence of local tumor recurrence.

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          Most cited references22

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          Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation.

          Primary cardiac and pericardial neoplasms are rare lesions and include both benign and malignant histologic types. Myxoma is the most frequent primary cardiac neoplasm, but other benign tumors include papillary fibroelastoma, rhabdomyoma, fibroma, hemangioma, lipoma, and paraganglioma. Cardiac sarcoma represents the second most common primary cardiac neoplasm. Lymphoma can also affect the heart primarily. Pericardial tumors that affect the heart include benign teratomas and malignant mesotheliomas. Patients affected with cardiac or pericardial neoplasms often present with cardiovascular compromise or embolic phenomena and exhibit cardiomegaly at chest radiography. Benign cardiac tumors typically manifest as intracavitary, mural, or epicardial focal masses, whereas malignant tumors demonstrate invasive features and may involve the heart diffusely. Benign lesions can usually be successfully excised, but patients with malignant lesions have an extremely poor prognosis.
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            Primary cardiac tumors.

            Cardiac tumors are a rare, but potentially curably form of heart disease. A high index of clinical suspicion is necessary for diagnosis as these tumors have protean manifestations that mimic a variety of other cardiac and noncardiac diseases. Presently, M-mode and two-dimensional echocardiography are utilized as safe, reliable, and noninvasive imaging modalities. Seventy-five per cent of these tumors are benign, with myxoma accounting for 50% and rhabodomyoma comprising 20% of lesions. Various histologic types of sarcoma are the predominant malignant cardiac neoplasms. With strict attention to avoiding perioperative tumor embolization, surgical resection of these lesions can be accomplished with minimal morbidity and mortality. Sixteen consecutive primary tumors of the heart have been surgically treated at Duke University Medical Center since 1966 with no perioperative deaths and no late recurrences.
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              CT and MR imaging of primary cardiac malignancies.

              Primary cardiac malignancies are rare tumors that are difficult to diagnose clinically. Different primary cardiac malignancies may have different clinical, morphologic, and radiologic features and intracardiac locations. Angiosarcoma is the most common primary cardiac malignancy. It tends to occur in the right atrium and involve the pericardium. Because of its tendency to hemorrhage, angiosarcoma often demonstrates areas of increased signal intensity with T1-weighted sequences. Undifferentiated sarcomas typically occur in the left atrium and have variable epidemiologic and radiologic features. Rhabdomyosarcoma is the most common primary cardiac malignancy in children and is more likely than other primary cardiac sarcomas to involve the valves. Primary cardiac osteogenic sarcoma almost always occurs in the left atrium and frequently demonstrates calcification. Certain features (eg, broad base of attachment, origin at a site other than the atrial septum) help differentiate this tumor from left atrial myxoma. Leiomyosarcoma favors the left atrium and tends to invade the pulmonary veins and mitral valve. Fibrosarcoma also tends to occur in the left atrium and is often necrotic. Liposarcoma is very rare and usually manifests as a large, infiltrating mass. Foci of macroscopic fat are occasionally seen. Primary cardiac lymphoma occurs more commonly in immunocompromised patients, frequently involves the pericardium, and, unlike other primary cardiac malignancies, may respond to chemotherapy. The advent of cross-sectional imaging has allowed earlier detection of primary cardiac malignancies as well as more accurate diagnosis and characterization.
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                Author and article information

                Journal
                J Cardiothorac Surg
                Journal of Cardiothoracic Surgery
                BioMed Central
                1749-8090
                2011
                9 April 2011
                : 6
                : 47
                Affiliations
                [1 ]Department of Cardiothoracic Surgery, University Medical Center Groningen, the Netherlands
                [2 ]Department of Cardiology, University Medical Center Groningen, the Netherlands
                [3 ]Department of Radiology, University Medical Center Groningen, the Netherlands
                [4 ]Department of Pathology, University Medical Center Groningen, the Netherlands
                Article
                1749-8090-6-47
                10.1186/1749-8090-6-47
                3090329
                21477334
                f37f8ffb-f216-445d-b3aa-d43f648f7800
                Copyright ©2011 Bouma et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 6 December 2010
                : 9 April 2011
                Categories
                Case Report

                Surgery
                Surgery

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