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      Cardiovascular magnetic resonance and PET-CT of left atrial paraganglioma

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          Abstract

          Cardiac paragangliomas are among the rarest primary cardiac tumors. We present a case of left atrial paraganglioma in a patient who presented with symptoms and signs of catecholamine excess in which cardiovascular magnetic resonance in multiple orientations and PET-CT played an important role in the diagnosis and tissue characterization.

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          Most cited references13

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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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            CT and MR imaging of benign primary cardiac neoplasms with echocardiographic correlation.

            Benign primary cardiac neoplasms are rare but may cause significant morbidity and mortality. However, they are usually treatable and can often be diagnosed with echocardiography, computed tomography (CT), or magnetic resonance (MR) imaging. Myxomas typically arise from the interatrial septum from a narrow base of attachment. Fibroelastomas are easily detected at echocardiography as small, mobile masses attached to valves by a short pedicle. Cardiac fibromas manifest as a large, noncontractile, solid mass in a ventricular wall at echocardiography and as a homogeneous mass with soft-tissue attenuation at CT. They are usually homogeneous and hypointense on T2-weighted MR images and isointense relative to muscle on T1-weighted images. Paragangliomas usually appear as large, echogenic left atrial masses at echocardiography and as circumscribed, heterogeneous masses with low attenuation at CT. These tumors are usually markedly hyperintense on T2-weighted MR images and iso- or hypointense relative to myocardium on T1-weighted images. Cardiac lipomas manifest at CT as homogeneous, low-attenuation masses in a cardiac chamber or in the pericardial space and demonstrate homogeneous increased signal intensity that decreases with fat-saturated sequences at T1-weighted MR imaging. Cardiac lymphangiomas manifest as cystic masses at echocardiography and typically demonstrate increased signal intensity at T1- and T2-weighted MR imaging. Familiarity with these imaging features and with the relative effectiveness of these modalities is essential for prompt diagnosis and effective treatment.
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              An overview of pheochromocytoma: history, current concepts, vagaries, and diagnostic challenges.

              W Manger (2006)
              Tragically as many as 50% of pheochromocytomas are discovered at autopsy, mainly because the diagnosis of this neuroendocrine tumor was not considered. Missing the diagnosis almost invariably results in devastating cardiovascular complications or death. Clinicians must always think of pheochromocytoma whenever evaluating a patient with sustained or paroxysmal hypertension or any manifestations suggesting hypercatecholaminemia. Very rarely, familial pheochromocytomas may cause no hypertension, symptoms, or signs. But biochemical testing can always establish the presence or absence of a pheochromocytoma, and localization with magnetic resonance imaging, computed tomography, or 131I or 123I-MIBG is almost always possible.
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                Author and article information

                Journal
                J Cardiovasc Magn Reson
                Journal of Cardiovascular Magnetic Resonance
                BioMed Central
                1097-6647
                1532-429X
                2010
                4 January 2010
                : 12
                : 1
                : 1
                Affiliations
                [1 ]Department of Radiological Sciences, University of California at Los Angeles, USA
                [2 ]Department of Pathology, University of California at Los Angeles, USA
                Article
                1532-429X-12-1
                10.1186/1532-429X-12-1
                2817869
                20047692
                5c6b6bd3-8e03-4c30-b9cc-be9cb670a3d9
                Copyright ©2010 Tomasian 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
                : 17 June 2009
                : 4 January 2010
                Categories
                Case report

                Cardiovascular Medicine
                Cardiovascular Medicine

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