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• W2110491119 abstract "HomeCirculationVol. 113, No. 12Diffuse Infiltration of Lymphoma of the Myocardium Mimicking Clinical Hypertrophic Cardiomyopathy Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessReview ArticlePDF/EPUBDiffuse Infiltration of Lymphoma of the Myocardium Mimicking Clinical Hypertrophic Cardiomyopathy P.W. Lee, K.S. Woo, Louis T.C. Chow, H.K. Ng, Wilson W.M. Chan, C.M. Yu and Anthony W.I. Lo P.W. LeeP.W. Lee From the Departments of Medicine and Therapeutics (P.W.L., K.S.W., W.W.M.C., C.M.Y.) and Anatomical and Cellular Pathology (L.T.C.C., H.K.N., A.W.I.L.), the Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong SAR, China. Search for more papers by this author , K.S. WooK.S. Woo From the Departments of Medicine and Therapeutics (P.W.L., K.S.W., W.W.M.C., C.M.Y.) and Anatomical and Cellular Pathology (L.T.C.C., H.K.N., A.W.I.L.), the Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong SAR, China. Search for more papers by this author , Louis T.C. ChowLouis T.C. Chow From the Departments of Medicine and Therapeutics (P.W.L., K.S.W., W.W.M.C., C.M.Y.) and Anatomical and Cellular Pathology (L.T.C.C., H.K.N., A.W.I.L.), the Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong SAR, China. Search for more papers by this author , H.K. NgH.K. Ng From the Departments of Medicine and Therapeutics (P.W.L., K.S.W., W.W.M.C., C.M.Y.) and Anatomical and Cellular Pathology (L.T.C.C., H.K.N., A.W.I.L.), the Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong SAR, China. Search for more papers by this author , Wilson W.M. ChanWilson W.M. Chan From the Departments of Medicine and Therapeutics (P.W.L., K.S.W., W.W.M.C., C.M.Y.) and Anatomical and Cellular Pathology (L.T.C.C., H.K.N., A.W.I.L.), the Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong SAR, China. Search for more papers by this author , C.M. YuC.M. Yu From the Departments of Medicine and Therapeutics (P.W.L., K.S.W., W.W.M.C., C.M.Y.) and Anatomical and Cellular Pathology (L.T.C.C., H.K.N., A.W.I.L.), the Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong SAR, China. Search for more papers by this author and Anthony W.I. LoAnthony W.I. Lo From the Departments of Medicine and Therapeutics (P.W.L., K.S.W., W.W.M.C., C.M.Y.) and Anatomical and Cellular Pathology (L.T.C.C., H.K.N., A.W.I.L.), the Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong SAR, China. Search for more papers by this author Originally published28 Mar 2006https://doi.org/10.1161/CIRCULATIONAHA.105.576306Circulation. 2006;113:e662–e664A 51-year-old man with good health previously presented with a 1-month history of shortness of breath on exertion. Transthoracic echocardiography revealed marked biventricular hypertrophy, mild mitral regurgitation, and moderate tricuspid regurgitation, suggestive of hypertrophic cardiomyopathy (Figure 1A and 1B; Movie I and Movie II). In the parasternal short-axis view, thickening of the left atrial wall and para-aortic tissue were evident, which is unusual in typical hypertrophic cardiomyopathy, a disease that primarily affects the ventricular myocardium (Figure 1C and Movie III). Computerized tomographic arteriography revealed no coronary artery disease. His heart failure symptoms worsened, and atrial fibrillation developed. At the same time, maculopapular skin rash developed on the upper trunk and the head and neck region. Hypercalcemia with an adjusted serum calcium level greater than 4 mmol/L was recorded. Other blood chemistry and hematologic examinations were unremarkable. His conditions deteriorated rapidly and, despite maximal supportive measures, he died 1 week after hospitalization. Limited clinical postmortem examination revealed a well-built gentleman with a heart weighing 790 g (Figure 2A). The walls of all 4 chambers of the heart were diffusely thickened by tan-colored infiltrative growth. The left and right ventricular walls were 2.5 and 1.5 cm thick, respectively (Figure 2B). The cross-sectional area of the superior vena cava was significantly reduced, with nodules resulting from subendothelial infiltrate. The leaflets of valves were not involved. Mild atherosclerosis was seen in the coronary arteries and major vessels. About 50 mL of straw-colored pericardial effusion was also noted. The maculopapular skin rash was distributed over the head and neck region, extending down to the umbilicus. Hemorrhage is seen superficially at the skin lesions. Microscopic examination revealed that diffuse large B-cell lymphoma (DLBCL) had infiltrated the myocardium and replaced a substantial amount of the cardiac muscle bulk (Figure 2C). The atypical lymphoid cells were large- to medium-sized, with abundant basophilic cytoplasm and large, moderately pleomorphic nuclei (Figure 2D). Prominent, centrally located nucleoli were also noted. These atypical lymphoid cells were immunoreactive to CD20 and were negative to T-cell markers such as CD3, CD5, and CD10. Epstein-Barr virus–encoding small nuclear RNA was not detected. Frank myocardial infarction was not detected in the residual myocardium, although contractile band necrosis can be seen in individual cardiomyocytes (Figure 2D, arrows). Infiltration of atypical lymphocytes was also noted in the pericardium. In the skin, perifollicular and perivascular infiltrations of these lymphoma cells were also detected. Download figureDownload PowerPointFigure 1. Transthoracic echocardiogram of parasternal long-axis view (A) showing markedly thickened left ventricular wall and interventricular septum, mimicking hypertrophic cardiomyopathy. Parasternal short-axis view at mid-ventricular level (B) showed concentric left ventricular hypertrophy. Parasternal short-axis view at aortic valve level (C), however, showed thickening of tissue in the paraaortic region and the left atrial wall, revealing the possibility of an infiltrative disease.Download figureDownload PowerPointFigure 2. At autopsy, the heart is grossly enlarged (A and B, scale in centimeters) with diffuse lymphoma infiltrates of the myocardium (C, hematoxylin and eosin stain; magnification ×100). Atypical lymphoid cells are large, with moderately pleomorphic nuclei (D, hematoxylin and eosin stain; magnification ×400). Some of the residual cardiomyocytes showed contractile band necrosis (arrows).Cardiac involvement with lymphoma was the most significant clinical and pathological process in our patient. Our case fits into the broader clinical definition of primary cardiac lymphoma.1 The incidence of cardiac lymphoma in postmortem examination is low, with a reported range of 0.15% to 1%. Secondary cardiac infiltration from nodal lymphoma of the mediastinum appears to be more common in clinical practice.2 In the past, most cases were discovered in postmortem examination.3,4 Recently, case reports were dominated by antemortem diagnosis and chemotherapy.5 DLBCL accounts for 30% to 40% of all adult non-Hodgkin lymphomas. DLBCL is, however, the most common type of primary cardiac lymphoma. Eighty percent of the case reports and 60% of autopsy series revealed DLBCL.1Most cases of cardiac lymphoma are solid, infiltrative nodule tumors in 1 or multiple chambers of the heart. The right heart is the most common site of cardiac lymphoma. Lymphomatous infiltration of the pericardium is also seen in a number of cases.1,3,5 However, the diffuse lymphoma infiltration of the myocardium in all 4 chambers in our patient is unusual. Massive infiltration of lymphoma cells in the myocardium results in irregular thickening of the walls of the heart, mimicking classic hypertrophic cardiomyopathy. Hypertrophy of the ventricular septum, without dilation of the heart chambers, results in the characteristic hemodynamic disturbances.6 Although only a limited autopsy was performed on our patient, we saw that the disease in our patient has spread beyond the heart and was disseminated at the final stage.The clinical symptoms of primary cardiac lymphoma are nonspecific and are related to the location of the tumor bulk as well as the functional status of the heart. Clinical presentations are acute, including dyspnea, edema, arrhythmia, and pericardial effusion.1,4,5 In one autopsy series, cardiac symptoms were found to be more common in T-cell lymphomas. Silent infiltration is more frequent in B-cell lymphomas.4 The pathophysiology of such clinical differences in lineages of lymphomas is not fully understood.The diagnosis of DLBCL was attained by postmortem examination in our patient, who presented with end-stage disease. Transesophageal echocardiography, computed tomography, and magnetic resonance imaging have shown characteristic features of cardiac lymphoma.1,7 Because of the variability of the morphology of the lymphomatous infiltrates, however, image findings may be nonspecific. Chemotherapy is indicated in these patients after a diagnosis with histological confirmation is established. Extra care needs to be exercised in the early stage of treatment to prevent overrapid tumor lysis, which may lead to rupture of the heart.The online-only Data Supplement, which contains Movie I through Movie III, can be found at http://circ.ahajournals.org/cgi/ content/full/113/12/e662/DC1.DisclosuresNone.FootnotesCorrespondence to Dr Anthony Lo, Department of Anatomical and Cellular Pathology, The Chinese University of Hong Kong, Prince of Wales Hospital, 30-32 Ngan Shing Street, Shatin, NT, Hong Kong SAR, China. E-mail [email protected]References1 Rolla G, Calligaris-Cappio F, Burke AP. Cardiac lymphoma. In: Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC, eds. Pathology and Genetics of Tumors of the Lung, Pleura, Thymus and Heart. Lyon, France: IARC Press; 2004: 282–283.Google Scholar2 Liang R, Yu CM, Au WY, Choy CK, Kwong YL. Diagnosis in oncology: case 2: secondary lymphoma of the heart manifesting as intracavitary masses. J Clin Oncol. 2000; 18: 1998–1999.CrossrefMedlineGoogle Scholar3 Chim CS, Chan AC, Kwong YL, Liang R. Primary cardiac lymphoma. Am J Hematol. 1997; 54: 79–83.CrossrefMedlineGoogle Scholar4 Chinen K, Izumo T. Cardiac involvement by malignant lymphoma: a clinicopathologic study of 25 autopsy cases based on the WHO classification. Ann Hematol. 2005; 84: 498–505.CrossrefMedlineGoogle Scholar5 Ikeda H, Nakamura S, Nishimaki H, Masuda K, Takeo T, Kasai K, Ohashi T, Sakamoto N, Wakida Y, Itoh G. Primary lymphoma of the heart: case report and literature review. Pathol Int. 2004; 54: 187–195.CrossrefMedlineGoogle Scholar6 Wynne J, Braunwald E. Cardiomyopathy and myocarditis. In: Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL, editors. Harrison’s Principles of Internal Medicine. 16th ed. New York, NY: McGraw-Hill Co; 2004.Google Scholar7 Ryu SJ, Choi BW, Choe KO. CT and MR findings of primary cardiac lymphoma: report upon 2 cases and review. Yonsei Med J. 2001; 42: 451–456.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Chen J, Ahmed T, Ahmed T, Iragavarapu C, Ramlal R and Arbune A (2022) Multimodality Imaging Guided Diagnosis and Treatment Response Evaluation in a Patient with Lymphoma with Right Atrioventricular Involvement, Current Problems in Cardiology, 10.1016/j.cpcardiol.2022.101273, 47:9, (101273), Online publication date: 1-Sep-2022. Schober K, Fox P, Abbott J, Côté E, Luis‐Fuentes V, Matos J, Stern J, Visser L, Scollan K, Chetboul V, Schrope D, Glaus T, Santilli R, Pariaut R, Stepien R, Arqued‐Soubeyran V, Toaldo M, Estrada A, MacDonald K, Karlin E and Rush J (2022) Retrospective evaluation of hypertrophic cardiomyopathy in 68 dogs, Journal of Veterinary Internal Medicine, 10.1111/jvim.16402, 36:3, (865-876), Online publication date: 1-May-2022. Luo Z, Cheng J and Wang Y (2022) Cardiac Infiltration as the First Manifestation of Acute Lymphoblastic Leukemia: A Systematic Review, Frontiers in Oncology, 10.3389/fonc.2022.805981, 12 Fox J and Strauss H (2022) Imaging the Cardiovascular System in the Cancer Patient Nuclear Oncology, 10.1007/978-3-031-05494-5_29, (1729-1752), . Bombace S, My I, Francone M and Monti L (2021) Tumoral Phenocopies of Hypertrophic Cardiomyopathy: The Role of Cardiac Magnetic Resonance, Journal of Clinical Medicine, 10.3390/jcm10081683, 10:8, (1683) Fukui T, Ogasawara N and Hasegawa S (2021) Unique autopsy case of primary cardiac lymphoma, BMJ Case Reports, 10.1136/bcr-2021-242174, 14:3, (e242174), Online publication date: 1-Mar-2021. Strauss H and Fox J (2017) Imaging the Heart in the Cancer Patient Nuclear Oncology, 10.1007/978-3-319-26236-9_29, (1483-1510), . Strauss H and Fox J (2016) Imaging the Heart in the Cancer Patient Nuclear Oncology, 10.1007/978-3-319-26067-9_29-1, (1-28), . Kikuchi Y, Oyama-Manabe N, Manabe O, Naya M, Ito Y, Hatanaka K, Tsutsui H, Terae S, Tamaki N and Shirato H (2013) Imaging characteristics of cardiac dominant diffuse large B-cell lymphoma demonstrated with MDCT and PET/CT, European Journal of Nuclear Medicine and Molecular Imaging, 10.1007/s00259-013-2436-5, 40:9, (1337-1344), Online publication date: 1-Sep-2013. Fox J and Strauss H (2013) Imaging the Heart in the Cancer Patient Nuclear Oncology, 10.1007/978-0-387-48894-3_29, (763-781), . Haran M, Enakpene E and Lodha A (2012) Cardiac Mass and Hypertrophic Cardiomyopathy as Aggressive Presentation of Primary Cardiac Lymphoma: A Case Report, Journal of Cancer Therapy, 10.4236/jct.2012.33026, 03:03, (183-186), . Shin W, Kim S, Choe Y, Hyeon J, Kim J and Chang S (2012) Non-mass-forming Lymphoma of the Left Ventricle Mimicking Non-ischemic Cardiomyopathy on MR Imaging: A Case Report, Journal of the Korean Society of Magnetic Resonance in Medicine, 10.13104/jksmrm.2012.16.2.189, 16:2, (189), . Cho S, Lin C, Chen Y and Lin S (2010) Primary cardiac lymphoma mimicking atrial thrombus in a patient who underwent permanent pacemaker implantation, Annals of Hematology, 10.1007/s00277-010-1082-2, 90:6, (739-740), Online publication date: 1-Jun-2011. Trifunovic D, Vujisic-Tesic B, Vuckovic M, Ostojic M, Ristic A, Bogdanovic A, Mihaljevic B, Andjelic B, Perunicic-Jovanovic M and Antonic Z (2010) Multimodality Imaging in the Assessment of Cardiac Lymphoma Presented as New-Onset Atrial Fibrillation, Echocardiography, 10.1111/j.1540-8175.2009.01074.x, 27:3, (332-336), Online publication date: 1-Mar-2010. Bédard E, Becker A and Gatzoulis M (2010) Cardiac Tumours Paediatric Cardiology, 10.1016/B978-0-7020-3064-2.00054-0, (1055-1065), . Carter T, Pariaut R, Snook E and Evans D (2008) Multicentric Lymphoma Mimicking Decompensated Hypertrophic Cardiomyopathy in a Cat, Journal of Veterinary Internal Medicine, 10.1111/j.1939-1676.2008.0208.x, 22:6, (1345-1347), Online publication date: 1-Nov-2008. March 28, 2006Vol 113, Issue 12 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.105.576306PMID: 16567575 Originally publishedMarch 28, 2006 PDF download Advertisement SubjectsEchocardiography" @default.
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