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• W2075311611 abstract "HomeCirculationVol. 129, No. 3An Unusual Case of Cardiomegaly Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessResearch ArticlePDF/EPUBAn Unusual Case of Cardiomegaly Antonia Delgado-Montero, MD, Covadonga Fernández-Golfín, MD, Laura Morán, MD, José M. Garrido, MD, Blanca Romera, MD, Alicia Megías, MD, Ana Gutierrez Pecharromán, MD, Amparo Benito, MD, María Eugenia Olmedo, MD, Miguel Castillo, MD and Jose L. Zamorano, MD Antonia Delgado-MonteroAntonia Delgado-Montero From the Departments of Cardiology (A.D.-M., C.F.-G., L.M., A.M., M.C., J.L.Z.), Cardiac Surgery (J.M.G.), Radiology (B.R.), Pathology (A.G.P., A.B.), and Oncology (M.E.O.), University Hospital Ramón y Cajal, Madrid, Spain. Search for more papers by this author , Covadonga Fernández-GolfínCovadonga Fernández-Golfín From the Departments of Cardiology (A.D.-M., C.F.-G., L.M., A.M., M.C., J.L.Z.), Cardiac Surgery (J.M.G.), Radiology (B.R.), Pathology (A.G.P., A.B.), and Oncology (M.E.O.), University Hospital Ramón y Cajal, Madrid, Spain. Search for more papers by this author , Laura MoránLaura Morán From the Departments of Cardiology (A.D.-M., C.F.-G., L.M., A.M., M.C., J.L.Z.), Cardiac Surgery (J.M.G.), Radiology (B.R.), Pathology (A.G.P., A.B.), and Oncology (M.E.O.), University Hospital Ramón y Cajal, Madrid, Spain. Search for more papers by this author , José M. GarridoJosé M. Garrido From the Departments of Cardiology (A.D.-M., C.F.-G., L.M., A.M., M.C., J.L.Z.), Cardiac Surgery (J.M.G.), Radiology (B.R.), Pathology (A.G.P., A.B.), and Oncology (M.E.O.), University Hospital Ramón y Cajal, Madrid, Spain. Search for more papers by this author , Blanca RomeraBlanca Romera From the Departments of Cardiology (A.D.-M., C.F.-G., L.M., A.M., M.C., J.L.Z.), Cardiac Surgery (J.M.G.), Radiology (B.R.), Pathology (A.G.P., A.B.), and Oncology (M.E.O.), University Hospital Ramón y Cajal, Madrid, Spain. Search for more papers by this author , Alicia MegíasAlicia Megías From the Departments of Cardiology (A.D.-M., C.F.-G., L.M., A.M., M.C., J.L.Z.), Cardiac Surgery (J.M.G.), Radiology (B.R.), Pathology (A.G.P., A.B.), and Oncology (M.E.O.), University Hospital Ramón y Cajal, Madrid, Spain. Search for more papers by this author , Ana Gutierrez PecharrománAna Gutierrez Pecharromán From the Departments of Cardiology (A.D.-M., C.F.-G., L.M., A.M., M.C., J.L.Z.), Cardiac Surgery (J.M.G.), Radiology (B.R.), Pathology (A.G.P., A.B.), and Oncology (M.E.O.), University Hospital Ramón y Cajal, Madrid, Spain. Search for more papers by this author , Amparo BenitoAmparo Benito From the Departments of Cardiology (A.D.-M., C.F.-G., L.M., A.M., M.C., J.L.Z.), Cardiac Surgery (J.M.G.), Radiology (B.R.), Pathology (A.G.P., A.B.), and Oncology (M.E.O.), University Hospital Ramón y Cajal, Madrid, Spain. Search for more papers by this author , María Eugenia OlmedoMaría Eugenia Olmedo From the Departments of Cardiology (A.D.-M., C.F.-G., L.M., A.M., M.C., J.L.Z.), Cardiac Surgery (J.M.G.), Radiology (B.R.), Pathology (A.G.P., A.B.), and Oncology (M.E.O.), University Hospital Ramón y Cajal, Madrid, Spain. Search for more papers by this author , Miguel CastilloMiguel Castillo From the Departments of Cardiology (A.D.-M., C.F.-G., L.M., A.M., M.C., J.L.Z.), Cardiac Surgery (J.M.G.), Radiology (B.R.), Pathology (A.G.P., A.B.), and Oncology (M.E.O.), University Hospital Ramón y Cajal, Madrid, Spain. Search for more papers by this author and Jose L. ZamoranoJose L. Zamorano From the Departments of Cardiology (A.D.-M., C.F.-G., L.M., A.M., M.C., J.L.Z.), Cardiac Surgery (J.M.G.), Radiology (B.R.), Pathology (A.G.P., A.B.), and Oncology (M.E.O.), University Hospital Ramón y Cajal, Madrid, Spain. Search for more papers by this author Originally published21 Jan 2014https://doi.org/10.1161/CIRCULATIONAHA.112.000099Circulation. 2014;129:396–398IntroductionA 64-year-old woman presented to the emergency department because of progressive fatigue and weight loss for the previous 3 months. She had a 10 pack-year history of smoking with no other significant medical history. Physical examination was unremarkable with normal cardiac and pulmonary auscultation. Her electrocardiogram showed sinus rhythm with very low voltages in all leads (Figure 1A). A chest x-ray film showed enlargement of the cardiac silhouette with normal pulmonary vascularization (Figure 1B). A transthoracic echocardiography was performed that revealed an homogeneous mass next to the right side of the heart without clear infiltration and moderate pericardial effusion (Figure 1C; see Movies I through III in the online-only Data Supplement). A contrast-enhanced full-body computed tomography showed a 12 × 8 cm soft-tissue density mass with some necrotic areas in the anterior and medium mediastinum (Figure 1D). No clear pericardial infiltration or involvement was noted, but small pleural and pericardial effusion was present. To better characterize the tumor, a cardiac magnetic resonance was performed, showing an 11 × 8.5 cm intrapericardial mass, with well-defined borders and no signs of pericardial or myocardial infiltration. Partial right atrium and superior vena cava compression was noted, without significant hemodynamic disturbance. T1-weighted fast spin-echo sequence showed multiple hyperintense subcentimetric areas that were hypointense in T2-weighted sequences. There were likely fibrous hypointense septa in both T1- and T2-weighted images (Figure 1E through 1G; see Movies IV and V in the online-only Data Supplement). Initial differential diagnosis was lymphoma, teratoma, or thymoma. A computed tomography–guided biopsy was made, and the histological examination confirmed the diagnosis of a cortical subtype (World Health Organization type B1) thymoma (Figure 1H). A combined chemotherapy treatment (cisplatin, doxorubicin, and cyclophosphamide) was initiated with no significant response. The patient developed recurrent pericardial effusion and pericardial tamponade, so a surgical approach was undertaken. Surgery was consistent with diagnosis of intrapericardial tumor with no pericardial or myocardial infiltration observed (Figure 2A). Partial resection of the mass was performed. A control cardiac magnetic resonance 2 months after surgery showed a 48 × 32 mm tumoral mass posterior to the main pulmonary artery, consistent with a nonexcised tumor. No other tumoral component was depicted (Figure 2B through 2F). The patient remains asymptomatic after 4 months of follow-up.Download figureDownload PowerPointFigure 1. A, Electrocardiogram showing low voltage in all leads. B, Posteroanterior chest radiograph showing cardiomegaly. C, Two-dimensional echocardiogram obtained in parasternal long-axis view showing an homogenous mass next to the right side of the heart. D, Full-body computed tomography in a sagittal view showing a soft-tissue density mass in the anterior and medium mediastinum with no clear pericardial visualization. E, T1-weighted fast spin echo in the axial plane showing an homogeneous 11 × 8.5 cm iso/hypointense mass anterior to the right ventricular outflow tract and right ventricle. Fat-plane separation is seen without evidence of pericardial or myocardial infiltration. F, Spectral presaturation with inversion recovery sequence showing hyperintense mass without fat component. G, T1-weighted fast spin echo post-Gadolinium in which light enhancement is seen. H, The mediastinal biopsy showed a tumor with thymic oval cells with low-grade cellular atypia and plenty of lymphocytic T cells (×20 magnification). The stain with cytokeratin AE1/AE3 highlight the thymic epithelial cells.Download figureDownload PowerPointFigure 2. A, Surgical resection of the intrapericardial thymoma (view from a standard median sternotomy). *shows the intrapericardial tumor, and ** shows the pericardial edges. B, T1-weighted fast spin echo in the axial plane after surgical resection showing tumor absence next to the right heart chambers. C and D, T1-weighted fast spin echo in axial plane at the level of great vessels showing the remaining tumoral mass posterior to the main pulmonary artery. E, Cine cardiac magnetic resonance image (gradient echo sequence) in a 4-chamber view showing small pericardial effusion with no compression of the right cardiac chambers. F, Cine cardiac magnetic resonance image (gradient echo sequence) in a 2-chamber view showing the postsurgical remaining tumoral mass inferior to the left pulmonary artery.Thymoma and thymic carcinomas are rare epithelial tumors, mainly detected in anterior or anterosuperior mediastinum. Clinical findings vary from asymptomatic patients (50% of the cases) to compression of adjacent structures and paraneoplastic syndromes, such as myastenia gravis, pure red cell aplasia, or immunodeficiency.1 Heart and great vessels are occasionally affected by orthotopic thymomas when they extend from the thymic cell in the anterior mediastinum. They are diagnosed very often after an incidental finding on chest radiography, but many cases require a multimodal approach. In our case, echocardiography made the initial diagnosis of the mass, confirmed on computed tomography, but only cardiac magnetic resonance was able to clearly delineate intrapericardial location and rule out pericardial or myocardial infiltration. As a rule, echocardiographic examination and especially cardiac magnetic resonance are essential when cardiac involvement is present as illustrated in the present patient. Although very rare, primary intrapericardial thymomas and thymic carcinomas have been described, originating from thymic cells that migrated to the pericardium during the embryonic development. There have been 6 other cases reported in the literature. Other reported ectopic sites for thymic cell tumors have been cervical, middle, or inferior mediastinum, diaphragm, or pleura.2 The prognosis varies widely depending on the tumor histology (classified by the World Health Organization) and the disease stage, which is determined by clinical and histopathologic findings.DisclosuresNone.FootnotesThe online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.112.000099/-/DC1.Correspondence to Antonia Delgado-Montero, MD, Cardiology Department, University Hospital Ramón y Cajal, Carretera Colmenar km 9.100, 28034 Madrid, Spain. E-mail [email protected]References1. Müller-Hermelink HK, Marx A. Thymoma.Curr Opin Oncol. 2000; 12:426–433.CrossrefMedlineGoogle Scholar2. Azoulay S, Adem C, Gatineau M, Finet JF, Bakdach H, Regnard JF, Capron F. Pericardial ectopic thymoma.Virchows Arch. 2005; 446:185–188.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetails January 21, 2014Vol 129, Issue 3 Advertisement Article InformationMetrics © 2014 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.112.000099PMID: 24446410 Originally publishedJanuary 21, 2014 PDF download Advertisement SubjectsComputerized Tomography (CT)" @default.
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