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      Primary right atrium angiosarcoma mimicking pericarditis

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          Primary cardiac neoplasms occur rarely and most of them are benign. Malignant tumors including angiosarcoma are extremely rare and have a non specific clinical presentation and a poor prognosis.

          Case presentation

          We present a case of a young male who was transferred to our hospital because of shock and multiple organ failure after a complicated pericardial biopsy. During the previous seven months he presented with recurrent episodes of pericardial effusions and tamponade. Chest computed tomography revealed a mass in the right atrium, infiltrating the myocardium and pericardium. During emergency surgery that followed, the patient died because of uncontrolled hemorrhage. Autopsy revealed the mass of the right atrium, which was identified on histological examination as primary cardiac angiosarcoma.


          This case highlights the difficulties both in early diagnosis and in the management of patients with cardiac angiosarcoma.

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          Most cited references 11

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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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            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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              Echocardiographic and pathologic characteristics of primary cardiac tumors: a study of 149 cases.

              To investigate the characteristics and pathological features of primary cardiac tumors and to evaluate the diagnostic sensitivity of echocardiography in primary cardiac tumors, all pathologic and echocardiographic records at the Chinese PLA general hospital and its satellite hospitals between January 1st, 1990 and January 1st, 2000 were reviewed to identify patients with a confirmed diagnosis of primary cardiac tumors. A total of 149 patients who had complete echocardiographic records and who were diagnosed with primary cardiac tumors were included in the study. Pathologic and echocardiographic records were reviewed retrospectively to evaluate the presence, location and histologic type of the tumors. The majority (n=118, 79.2%) of cases had been diagnosed with benign tumors. Myxoma was the most common histologic type accounting for 50.0% of total cardiac tumors. Lipoma was the second most common type of benign tumor. Among cases with malignant tumors (n=31, 20.8%), unclassified sarcoma (n=7), angiosarcoma (n=6) and rhabdomyosarcoma (n=6) were the common histologic types of primary malignant tumor. Non-myxomatous benign tumors were more likely to have occurred in the ventricle than myxomas (17/43, 39.5% vs. 7/75, 9.3%; P=0.00). The proportion of pericardium involvement in the malignant tumors (8/31, 25.8%) was significantly higher than that in the myxomas (0/75, 0%; P=0.00) and non-myxomas (2/43, 4.7%; P=0.01). The diagnostic sensitivity of transthoracic and transesophageal echocardiography was 93.3% (139/149) and 96.8% (30/31), respectively. The study, using a relatively large sample, confirms that myxoma was the most common primary cardiac tumor. The locations of tumor involvement varied by types of tumor. Echocardiography may be a useful tool for early diagnosis of primary cardiac tumors.

                Author and article information

                World J Surg Oncol
                World Journal of Surgical Oncology
                BioMed Central
                22 October 2007
                : 5
                : 120
                [1 ]Department of Critical Care, Medical School of Athens University, Evangelismos Hospital, Athens Greece
                [2 ]Department of Radiology, Evangelismos Hospital, Athens, Greece
                [3 ]Department of Cardiothoracic Surgery, Evangelismos Hospital, Athens, Greece
                [4 ]Department of Pathology, Evangelismos Hospital, Athens, Greece
                Copyright © 2007 Kontogiorgi 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.

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