Unusual Location for a Large Cardiac Fibroma
2011; Lippincott Williams & Wilkins; Volume: 124; Issue: 13 Linguagem: Inglês
10.1161/circulationaha.111.018374
ISSN1524-4539
AutoresAlbert Teis, Mary N. Sheppard, Francisco Alpendurada,
Tópico(s)Cardiac tumors and thrombi
ResumoHomeCirculationVol. 124, No. 13Unusual Location for a Large Cardiac Fibroma Free AccessBrief ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessBrief ReportPDF/EPUBUnusual Location for a Large Cardiac Fibroma Albert Teis, MD, Mary N. Sheppard, MD and Francisco Alpendurada, MD Albert TeisAlbert Teis From the Cardiovascular MR Department (A.T., F.A.) and Histopathology Department (M.N.S.), Royal Brompton Hospital, London, United Kingdom. , Mary N. SheppardMary N. Sheppard From the Cardiovascular MR Department (A.T., F.A.) and Histopathology Department (M.N.S.), Royal Brompton Hospital, London, United Kingdom. and Francisco AlpenduradaFrancisco Alpendurada From the Cardiovascular MR Department (A.T., F.A.) and Histopathology Department (M.N.S.), Royal Brompton Hospital, London, United Kingdom. Originally published27 Sep 2011https://doi.org/10.1161/CIRCULATIONAHA.111.018374Circulation. 2011;124:1481–1482A 37-year-old woman with palpitations and chest discomfort was referred to our institution for assessment of a right ventricular (RV) mass seen on echocardiography (Figure 1). Cardiovascular magnetic resonance confirmed the presence of a large oval mass (6×7 cm) within the basal RV free wall (online-only Data Supplement Movies I and II). The mass was causing compression of the RV inlet but no invasion of the tricuspid annulus or valve (Figure 2). The mass had a low signal on T1- and T2-weighted images, no increase in signal during first-pass perfusion, but diffuse delayed hyperenhancement after gadolinium injection, with some central areas of relatively low signal (Figure 2, B through D). These features are typical of cardiac fibroma. On surgery, a large, lobulated solid mass was excised from the RV free wall (Figure 3). Histological analysis with hematoxylin-and-eosin staining showed dense whorled areas with bland spindle cells and cardiac myocytes at the edge (Figure 4A). Elastin–Van Gieson trichrome staining revealed a profusion of collagen admixed with elastin fibers (Figure 4B). The spindle cells were immunopositive for smooth muscle actin, which confirmed fibroma (Figure 4C).Download figureDownload PowerPointFigure 1. Transthoracic echocardiography images demonstrate a large oval mass (m) attached to the right ventricular (RV) free wall close to the auriculoventricular groove on parasternal (A), apical 4-chamber (B), and subcostal (C) views. Ao indicates aorta; LV, left ventricle.Download figureDownload PowerPointFigure 2. Cardiovascular magnetic resonance image showing a large oval mass within the basal right ventricular free wall (A), with low signal on T1-weighted (B) and T2-weighted (C) spin-echo images. There is diffuse and intense enhancement in the late phase after gadolinium injection, with some central areas of relatively low signal (D).Download figureDownload PowerPointFigure 3. Macroscopic views of the large excised mass. Anterior (A) and posterior (B) views of the external surface, and interior aspect (C and D) of the mass after section of the piece in two parts.Download figureDownload PowerPointFigure 4. Microscopic analysis. Hematoxylin-and-eosin staining (A) showing bland spindle cells (asterisk) and cardiac myocytes at the edge (arrow). Elastic-Van Gieson trichrome staining (B) shows collagen (pink) admixed with elastin fibers (dark blue). Actin α-smooth muscle immunohistology analysis (C) revealed that spindle cells were positive for smooth muscle actin (brown).Cardiac fibromas commonly present in infancy and adolescence and frequently involve the interventricular septum and left ventricular free wall. Involvement of the right side of the heart by fibromas is uncommon, and differential diagnoses include other primary benign tumors (such as myxoma, lipoma, rhabdomyoma, and hemangioma) and malignant tumors (such as sarcomas, lymphomas, and cardiac metastases). On magnetic resonance imaging, fibroma is the only intramural tumor to have a combination of an intermediate-low signal on T1-weighted images plus a low signal on T2-weighted images, which reflects small intracellular and vascular space and low water content. Myxomas can also have an intermediate signal on T1 and a low signal on T2 images, but they are intracavitary in nature. A lipoma has a typically high T1 signal due to high fat content. Cardiac rhabdomyomas are frequently multiple and return an intermediate-high signal on T1 and an intermediate signal on T2 images. Hemangiomas are enhanced with first-pass perfusion and have an increased signal on T2 images (probably because of slow-flowing blood in the tumor vessels). Finally, malignant tumors tend to return a high signal on T2-weighted images as a result of increased water content due to cellular necrosis and tissue inflammation. In addition to the characteristics described above, the other differentiating feature of cardiac fibromas is delayed hyperenhancement after gadolinium injection, which is usually more intense than with any other tumor. This feature reflects contrast accumulation within an expanded extracellular compartment by deposition of collagen and elastin fibers (Figures 2D and 4).AcknowledgmentsThis work was supported by the NIHR Cardiovascular Biomedical Research Unit of Royal Brompton and Harefield NHS Foundation Trust and Imperial College London.DisclosuresNone.FootnotesThe online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161.CIRCULATIONAHA.111.018374/-/DC1.Correspondence to Albert Teis, MD, Cardiovascular MR Department, Royal Brompton Hospital, Sydney St, SW3 6NP, London, United Kingdom. E-mail [email protected]es Previous Back to top Next FiguresReferencesRelatedDetailsCited By Teng F, Yang S, Chen D, Fang W, Shang J, Dong S, Cui Y, Fu W, Zheng M, Li Y and Lian G (2022) Cardiac fibroma: A clinicopathologic study of a series of 12 cases, Cardiovascular Pathology, 10.1016/j.carpath.2021.107381, 56, (107381), Online publication date: 1-Jan-2022. Kimura A, Kanzaki H, Izumi C, Khan T, Bouzas-Mosquera A, Estabragh A, Mukherjee R and Green P (2020) A case report of primary cardiac fibroma: an effective approach for diagnosis and therapy of a pathologically benign tumour with an unfavourable prognosis, European Heart Journal - Case Reports, 10.1093/ehjcr/ytaa186, 4:4, (1-5), Online publication date: 1-Aug-2020. Yong M, Brink J and Zimmet A (2015) Right Ventricular Reconstruction After Resection of Cardiac Fibroma, Journal of Cardiac Surgery, 10.1111/jocs.12584, 30:8, (640-642), Online publication date: 1-Aug-2015. Chu Z, Zhu Z, Liu M and Lv F (2013) Cardiac Fibromas in the Adult, Journal of Cardiac Surgery, 10.1111/jocs.12251, 29:2, (159-162), Online publication date: 1-Mar-2014. Miglioranza M, Leiria T, Haertel J, Winkler M, Fernández-Golfin C and Zamorano J (2013) The Role of Three-Dimensional Echocardiography in Interventricular Mass Evaluation, Echocardiography, 10.1111/echo.12161, 30:5, (E125-E127), Online publication date: 1-May-2013. Chen Y, Sun J, Chen W, Peng Y and An Q (2013) Third-Degree Atrioventricular Block in an Adult With a Giant Cardiac Fibroma, Circulation, 10.1161/CIRCULATIONAHA.112.131417, 127:13, Online publication date: 2-Apr-2013. September 27, 2011Vol 124, Issue 13 Advertisement Article InformationMetrics © 2011 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.111.018374PMID: 21947936 Originally publishedSeptember 27, 2011 PDF download Advertisement SubjectsComputerized Tomography (CT)Imaging
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