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<front>
<journal-meta>
<journal-id journal-id-type="publisher-id">abcic</journal-id>
<journal-title-group>
<journal-title>ABC Imagem Cardiovascular</journal-title>
<abbrev-journal-title abbrev-type="publisher">ABC Imagem Cardiovasc.</abbrev-journal-title></journal-title-group>
<issn pub-type="epub">2675-312X</issn>
<issn pub-type="ppub">2318-8219</issn>
<publisher>
<publisher-name>Departamento de Imagem Cardiovascular da Sociedade Brasileira de Cardiolodia (DIC/SBC)</publisher-name></publisher>
</journal-meta>
<article-meta>
<article-id pub-id-type="doi">10.36660/abcimg.20250033i</article-id>
<article-id pub-id-type="publisher-id">abcimg.20250033i</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Case Report</subject>
</subj-group>
</article-categories>
<title-group>
<article-title>Turmoil of Symptoms: The Devastating Impact of Cardiac Lymphoma – Multiple Clinical Manifestations and the Lethal Causes of Cardiac Involvement</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0000-0001-7839-3965</contrib-id>
<name><surname>Carvalho</surname><given-names>Gustavo</given-names></name>
<role>Conception and design of the research</role>
<role>acquisition of data</role>
<role>analysis and interpretation of the data</role>
<role>writing of the manuscript</role>
<role>critical revision of the manuscript for intellectual content</role>
<xref ref-type="aff" rid="aff1"><sup>1</sup></xref><xref ref-type="corresp" rid="c1"/></contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0009-0008-0807-4107</contrib-id>
<name><surname>Carvalho</surname><given-names>Maria Fernanda Miranda</given-names></name>
<role>acquisition of data</role>
<role>analysis and interpretation of the data</role>
<role>writing of the manuscript</role>
<role>critical revision of the manuscript for intellectual content</role>
<role>review of references</role>
<xref ref-type="aff" rid="aff1"><sup>1</sup></xref></contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0000-0002-7232-4552</contrib-id>
<name><surname>Magalhães</surname><given-names>Tiago</given-names></name>
<role>acquisition of data</role>
<role>critical revision of the manuscript for intellectual content</role>
<role>magnetic resonance imaging</role>
<xref ref-type="aff" rid="aff1"><sup>1</sup></xref><xref ref-type="aff" rid="aff2"><sup>2</sup></xref></contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0000-0001-9014-2720</contrib-id>
<name><surname>Varela</surname><given-names>Alexandre Manoel</given-names></name>
<role>analysis and interpretation of the data</role>
<role>critical revision of the manuscript for intellectual content</role>
<xref ref-type="aff" rid="aff3"><sup>3</sup></xref></contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0009-0003-4528-4174</contrib-id>
<name><surname>Staszko</surname><given-names>Kamila Fernanda</given-names></name>
<role>acquisition of data</role>
<role>critical revision of the manuscript for intellectual content</role>
<role>echocardiogram images</role>
<xref ref-type="aff" rid="aff1"><sup>1</sup></xref></contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0000-0001-5246-0453</contrib-id>
<name><surname>Hayashida</surname><given-names>Mariana Rie</given-names></name>
<role>acquisition of data</role>
<role>critical revision of the manuscript for intellectual content</role>
<role>pathological anatomy analysis</role>
<xref ref-type="aff" rid="aff1"><sup>1</sup></xref></contrib>
<aff id="aff1">
<label>1</label>
<institution content-type="orgname">Universidade Federal do Paraná</institution>
<addr-line>
<named-content content-type="city">Curitiba</named-content>
<named-content content-type="state">PR</named-content>
</addr-line>
<country country="BR">Brazil</country>
<institution content-type="original">Universidade Federal do Paraná, Curitiba, PR – Brazil</institution>
</aff>
<aff id="aff2">
<label>2</label>
<institution content-type="orgname">Hospital do Coração</institution>
<addr-line>
<named-content content-type="city">São Paulo</named-content>
<named-content content-type="state">SP</named-content>
</addr-line>
<country country="BR">Brazil</country>
<institution content-type="original">Hospital do Coração, São Paulo, SP – Brazil</institution>
</aff>
<aff id="aff3">
<label>3</label>
<institution content-type="orgname">Hospital Erasto Gaertner</institution>
<addr-line>
<named-content content-type="city">Curitiba</named-content>
<named-content content-type="state">PR</named-content>
</addr-line>
<country country="BR">Brazil</country>
<institution content-type="original">Hospital Erasto Gaertner, Curitiba, PR – Brazil</institution>
</aff>
</contrib-group>
<author-notes>
<corresp id="c1"><label>Mailing Address:</label> <bold>Gustavo Carvalho</bold> • CHC UFPR. Dionira M Klemtz, 201, casa 107. Postal code: <postal-code>81320-390</postal-code>. Bairro Fazendinha, Curitiba, PR – Brazl E-mail: <email>gustavocarvalho1975@gmail.com</email></corresp>
<fn fn-type="conflict"><label>Potential Conflict of Interest</label>
<p>No potential conflict of interest relevant to this article was reported.</p></fn>
<fn fn-type="edited-by"><label>Editor responsible for the review:</label> <p>Marcelo Tavares</p></fn>
</author-notes>
<pub-date publication-format="electronic" date-type="pub">
<day>15</day>
<month>08</month>
<year>2025</year></pub-date>
<pub-date publication-format="electronic" date-type="collection">
<year>2025</year></pub-date>
<volume>38</volume>
<issue>3</issue>
<elocation-id>e20250033</elocation-id>
<history>
<date date-type="received">
<day>05</day>
<month>05</month>
<year>2025</year>
</date>
<date date-type="rev-recd">
<day>05</day>
<month>05</month>
<year>2025</year>
</date>
<date date-type="accepted">
<day>05</day>
<month>05</month>
<year>2025</year>
</date>
</history>
<permissions>
<license license-type="open-access" xlink:href="https://creativecommons.org/licenses/by/4.0/" xml:lang="en">
<license-p>This is an open-access article distributed under the terms of the Creative Commons Attribution License</license-p>
</license>
</permissions>
<abstract>
<title>Abstract</title>
<p>Primary cardiac lymphoma (PCL) is a rare and highly lethal neoplasm, whose nonspecific clinical presentation makes early diagnosis difficult. This article reports the case of an elderly patient, previously healthy, who experienced dizziness and syncope for four months before the discovery of a large tumor mass in the heart. Imaging exams, such as echocardiography and magnetic resonance imaging, were crucial for tumor identification, and the biopsy confirmed it to be a B-cell lymphoma with high replication. However, given the severity of the condition and the family&apos;s refusal to proceed with treatment, the patient progressed to sudden death two weeks after diagnosis. Primary cardiac tumors (PCTs) are underdiagnosed, with varied symptoms that may include vascular obstructions, arrhythmias, and heart failure. PCLs account for only 1% to 2% of primary cardiac masses and are more common in elderly men. The diagnosis is challenging due to the absence of specific signs, making imaging exams and biopsies essential for disease confirmation. This case illustrates the complexity of managing PCLs, whose rapid progression limits therapeutic options. The lack of specific symptoms and silent progression highlight the importance of early diagnosis. The high mortality rate reinforces the need for greater medical attention to this condition, which frequently leads to fatal outcomes before an effective intervention can be implemented.</p>
</abstract>
<kwd-group xml:lang="en">
<title>Keywords</title>
<kwd>Heart Neoplasms</kwd>
<kwd>Echocardiography</kwd>
<kwd>Cardiac Arrhythmias</kwd>
<kwd>Biopsy</kwd>
</kwd-group>
<counts>
<fig-count count="6"/>
<table-count count="0"/>
<equation-count count="0"/>
<ref-count count="12"/>
</counts>
</article-meta>
</front>
<body>
<sec sec-type="intro">
<title>Introduction</title>
<p>Primary cardiac tumors (PCTs) are rare entities in clinical practice and can be classified as benign lesions (myxomas) or malignant tumors (sarcomas and lymphomas).<sup><xref ref-type="bibr" rid="B1">1</xref>,<xref ref-type="bibr" rid="B2">2</xref></sup> With the increasing life expectancy of the population, a higher incidence of these tumors is expected, highlighting the need for greater attention to their possible symptoms, especially considering their varied clinical manifestations.<sup><xref ref-type="bibr" rid="B2">2</xref>,<xref ref-type="bibr" rid="B3">3</xref></sup></p>
<p>Considering this, it is important to emphasize that PCTs are often underdiagnosed, which may lead to fatal outcomes before an effective therapeutic approach can be implemented.<sup><xref ref-type="bibr" rid="B2">2</xref></sup> This article presents a case of primary myocardial lymphoma in an elderly patient, previously healthy, who experienced sudden death two weeks after diagnosis.</p>
<p>Thus, the objective of this study is to discuss the clinical and diagnostic characteristics of PCTs.</p>
</sec>
<sec sec-type="cases">
<title>Case report</title>
<p>A 78-year-old man, previously healthy, presented with dizziness and syncope worsening over four months. The patient had been seen at other emergency care units, but his symptoms progressively worsened.</p>
<p>On physical examination, no significant findings were noted. The electrocardiogram showed sinus rhythm with diffuse ventricular repolarization abnormalities. A chest X-ray revealed a cardiac silhouette at the upper limit of normal and bilateral costophrenic angle blunting. A transthoracic echocardiogram detected a large tumor mass infiltrating the interventricular septum and the inferolateral wall of the right ventricle, extending into the right atrial and ventricular cavities, as well as the left ventricular cavity adjacent to the septum (<xref ref-type="fig" rid="f1">Figure 1</xref>).</p>
<fig id="f1">
<label>Figure 1</label>
<caption><title>Transthoracic echocardiogram showing a tumor mass infiltrating the atrioventricular valves, the ventricular myocardium at the septal level, and the anterior and inferior walls of both ventricles. The tumor occupies the atrial and right ventricular cavities (arrows); pericardial effusion.</title></caption>
<graphic xlink:href="2675-312X-abcic-38-03-e20250033-gf01.tif"/>
</fig>
<p>Given this, the patient was referred for a magnetic resonance imaging scan for a more detailed assessment of the neoplasm, which confirmed the echocardiographic findings (<xref ref-type="fig" rid="f2">Figure 2</xref>). An endomyocardial biopsy was performed, guided by transthoracic echocardiography and fluoroscopy, conducted simultaneously in the hemodynamics room. The histopathological study and immunohistochemical analysis concluded that it was an invasive B-cell lymphoma with intense cellular replication (<xref ref-type="fig" rid="f3">Figure 3</xref>).</p>
<fig id="f2">
<label>Figure 2</label>
<caption><title>Magnetic resonance imaging showing the extent of cardiac involvement by lymphoma (*) demonstrated in the sagittal (A) and transverse (B) planes, along with pericardial and bilateral pleural effusion.</title></caption>
<graphic xlink:href="2675-312X-abcic-38-03-e20250033-gf02.tif"/>
</fig>
<fig id="f3">
<label>Figure 3</label>
<caption><title>Histopathological and immunohistochemical findings demonstrating B-cell lymphoma with intense cellular proliferation in Ki-67 (A), and B lymphocytes marked in CD20 (B).</title></caption>
<graphic xlink:href="2675-312X-abcic-38-03-e20250033-gf03.tif"/>
</fig>
<p>After a detailed explanation of this complex and severe clinical case to the patient and his family, they refused the implementation of any form of treatment and requested discharge to his home, where he remained asymptomatic for approximately two more weeks, before experiencing sudden death.</p>
</sec>
<sec sec-type="discussion">
<title>Discussion</title>
<p>Although reported since the 16th century, PCTs are rare in clinical practice, with an incidence ranging from 0.0017% to 0.03% in autopsy studies.<sup><xref ref-type="bibr" rid="B1">1</xref>-<xref ref-type="bibr" rid="B3">3</xref></sup> PCTs can primarily manifest as benign lesions, with an incidence rate of 75%, or as malignant tumors, accounting for 25% of cases.<sup><xref ref-type="bibr" rid="B3">3</xref>-<xref ref-type="bibr" rid="B5">5</xref></sup></p>
<p>On the other hand, primary cardiac lymphomas (PCLs) are classified as malignant neoplasms, representing 1% to 2% of all primary cardiac masses.<sup><xref ref-type="bibr" rid="B2">2</xref>-<xref ref-type="bibr" rid="B5">5</xref></sup></p>
<p>Although PCTs generally affect more women, PCLs are more common in men over 60 years old, which is well represented by the patient described in this case report.<sup><xref ref-type="bibr" rid="B4">4</xref>-<xref ref-type="bibr" rid="B6">6</xref></sup></p>
<p>PCLs often present nonspecific and multifaceted clinical manifestations.<sup>14</sup> Initially, the patients may be asymptomatic or oligosymptomatic, but as the disease progresses, they develop various signs and symptoms due to direct and/or indirect involvement of multiple organs and systems through heterogeneous, simultaneous, and diverse mechanisms. These include recurrent vascular obstructive phenomena caused by thrombi and/or tumor fragments, leading to stroke, peripheral and visceral arterial embolism, and pulmonary thromboembolism.<sup><xref ref-type="bibr" rid="B4">4</xref>-<xref ref-type="bibr" rid="B7">7</xref></sup></p>
<p>The tumor itself may cause fixed or transient intracardiac blood flow obstruction, depending on its size and morphology (intramural or pedunculated intracavitary), potentially compromising cardiac output.<sup><xref ref-type="bibr" rid="B2">2</xref>,<xref ref-type="bibr" rid="B4">4</xref></sup> When the myocardial electrical conduction system is affected, arrhythmia may occur, sometimes unexpectedly fatal, as possibly seen in the case presented here.<sup><xref ref-type="bibr" rid="B5">5</xref>-<xref ref-type="bibr" rid="B7">7</xref></sup></p>
<p>Thus, in cases of cardiac tumors, it is undeniable that, due to their nonspecific symptoms, establishing a timely diagnosis to apply effective therapy is a challenge for the healthcare team, as demonstrated in this report.<sup><xref ref-type="bibr" rid="B6">6</xref>,<xref ref-type="bibr" rid="B7">7</xref></sup> Furthermore, it is essential to highlight the importance of imaging exams, such as echocardiography and cardiac magnetic resonance imaging, in the detection of these tumor masses and in performing endomyocardial biopsies to establish a histopathological diagnosis.<sup><xref ref-type="bibr" rid="B8">8</xref>-<xref ref-type="bibr" rid="B12">12</xref></sup></p>
</sec>
<sec sec-type="conclusions">
<title>Conclusion</title>
<p>PCL represents a rare but extremely severe condition, whose nonspecific clinical presentation and silent progression make its diagnosis a major challenge.</p>
<p>As discussed in this report, its multiple manifestations can mask the true extent of the disease, resulting in a discouraging prognosis. Cardiac involvement by lymphoma not only compromises organ function but also exposes the patient to a high risk of lethal events, such as fatal arrhythmias and critical vascular obstructions.</p>
<p>Given this scenario, the importance of early and thorough investigation using imaging exams, histopathological aspects, and immunohistochemical analysis for diagnostic definition is fundamental. However, as evidenced in the case described, even with the diagnosis established, therapeutic options may be limited due to the rapid progression of the disease and the patient&apos;s own decisions.</p>
<p>Thus, the devastating impact of cardiac lymphoma extends beyond its physiological repercussions, encompassing therapeutic limitations and abrupt outcomes, emphasizing the need for greater medical-scientific attention and understanding for future approaches to this unusual and fatal entity.</p>
</sec>
</body>
<back>
<fn-group>
<fn fn-type="financial-disclosure" id="fn1"><label>Sources of Funding</label>
<p>There were no external funding sources for this study.</p></fn>
<fn fn-type="other" id="fn2"><label>Study Association</label>
<p>This study is not associated with any thesis or dissertation work.</p></fn>
<fn fn-type="other" id="fn3"><label>Ethics Approval and Consent to Participate</label>
<p>This study was approved by the Ethics Committee of the Hospital De Clínicas Da Universidade Federal Do Paraná under the protocol number 90118225.4.0000.0096. All the procedures in this study were in accordance with the 1975 Helsinki Declaration, updated in 2013. The Free and Informed Consent Form was waived.</p></fn>
<fn fn-type="other" id="fn4"><label>Use of Artificial Intelligence</label>
<p>The authors did not use any artificial intelligence tools in the development of this work.</p></fn>
</fn-group>
<sec sec-type="data-availability" specific-use="data-in-article">
<label>Availability of Research Data</label>
<p>The underlying content of the research text is contained within the manuscript.</p>
</sec>
<ref-list>
<title>References</title>
<ref id="B1"><label>1</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Leja</surname> <given-names>MJ</given-names></name> <name><surname>Shah</surname> <given-names>DJ</given-names></name> <name><surname>Reardon</surname> <given-names>MJ</given-names></name></person-group> <article-title>Primary Cardiac Tumors</article-title> <source>Tex Heart Inst J</source> <year>2011</year><volume>38</volume><issue>3</issue><fpage>261</fpage><lpage>262</lpage></element-citation>
<mixed-citation>Leja MJ, Shah DJ, Reardon MJ. Primary Cardiac Tumors. Tex Heart Inst J. 2011;38(3):261-2.</mixed-citation></ref>
<ref id="B2"><label>2</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Tyebally</surname> <given-names>S</given-names></name> <name><surname>Chen</surname> <given-names>D</given-names></name> <name><surname>Bhattacharyya</surname> <given-names>S</given-names></name> <name><surname>Mughrabi</surname> <given-names>A</given-names></name> <name><surname>Hussain</surname> <given-names>Z</given-names></name> <name><surname>Manisty</surname> <given-names>C</given-names></name> <etal/></person-group> <article-title>Cardiac Tumors: JACC CardioOncology State-of-the-Art Review</article-title> <source>JACC CardioOncol</source> <year>2020</year><volume>2</volume><issue>2</issue><fpage>293</fpage><lpage>311</lpage><pub-id pub-id-type="doi">10.1016/j.jaccao.2020.05.009</pub-id></element-citation>
<mixed-citation>Tyebally S, Chen D, Bhattacharyya S, Mughrabi A, Hussain Z, Manisty C, et al. Cardiac Tumors: JACC CardioOncology State-of-the-Art Review. JACC CardioOncol. 2020;2(2):293-311. doi: 10.1016/j.jaccao.2020.05.009.</mixed-citation></ref>
<ref id="B3"><label>3</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Cresti</surname> <given-names>A</given-names></name> <name><surname>Chiavarelli</surname> <given-names>M</given-names></name> <name><surname>Glauber</surname> <given-names>M</given-names></name> <name><surname>Tanganelli</surname> <given-names>P</given-names></name> <name><surname>Scalese</surname> <given-names>M</given-names></name> <name><surname>Cesareo</surname> <given-names>F</given-names></name> <etal/></person-group> <article-title>Incidence Rate of Primary Cardiac Tumors: A 14-Year Population Study</article-title> <source>J Cardiovasc Med</source> <year>2016</year><volume>17</volume><issue>1</issue><fpage>37</fpage><lpage>43</lpage><pub-id pub-id-type="doi">10.2459/JCM.0000000000000059</pub-id></element-citation>
<mixed-citation>Cresti A, Chiavarelli M, Glauber M, Tanganelli P, Scalese M, Cesareo F, et al. Incidence Rate of Primary Cardiac Tumors: A 14-Year Population Study. J Cardiovasc Med. 2016;17(1):37-43. doi: 10.2459/JCM.0000000000000059.</mixed-citation></ref>
<ref id="B4"><label>4</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Carras</surname> <given-names>S</given-names></name> <name><surname>Berger</surname> <given-names>F</given-names></name> <name><surname>Chalabreysse</surname> <given-names>L</given-names></name> <name><surname>Callet-Bauchut</surname> <given-names>E</given-names></name> <name><surname>Cordier</surname> <given-names>JF</given-names></name> <name><surname>Salles</surname> <given-names>G</given-names></name> <etal/></person-group> <article-title>Primary Cardiac Lymphoma: Diagnosis, Treatment and Outcome in a Modern Series</article-title> <source>Hematol Oncol</source> <year>2017</year><volume>35</volume><issue>4</issue><fpage>510</fpage><lpage>519</lpage><pub-id pub-id-type="doi">10.1002/hon.2301</pub-id></element-citation>
<mixed-citation>Carras S, Berger F, Chalabreysse L, Callet-Bauchut E, Cordier JF, Salles G, et al. Primary Cardiac Lymphoma: Diagnosis, Treatment and Outcome in a Modern Series. Hematol Oncol. 2017;35(4):510-9. doi: 10.1002/hon.2301.</mixed-citation></ref>
<ref id="B5"><label>5</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Sarjeant</surname> <given-names>JM</given-names></name> <name><surname>Butany</surname> <given-names>J</given-names></name> <name><surname>Cusimano</surname> <given-names>RJ</given-names></name></person-group> <article-title>Cancer of the Heart: Epidemiology and Management of Primary Tumors and Metastases</article-title> <source>Am J Cardiovasc Drugs</source> <year>2003</year><volume>3</volume><issue>6</issue><fpage>407</fpage><lpage>421</lpage><pub-id pub-id-type="doi">10.2165/00129784-200303060-00004</pub-id></element-citation>
<mixed-citation>Sarjeant JM, Butany J, Cusimano RJ. Cancer of the Heart: Epidemiology and Management of Primary Tumors and Metastases. Am J Cardiovasc Drugs. 2003;3(6):407-21. doi: 10.2165/00129784-200303060-00004.</mixed-citation></ref>
<ref id="B6"><label>6</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Butany</surname> <given-names>J</given-names></name> <name><surname>Nair</surname> <given-names>V</given-names></name> <name><surname>Naseemuddin</surname> <given-names>A</given-names></name> <name><surname>Nair</surname> <given-names>GM</given-names></name> <name><surname>Catton</surname> <given-names>C</given-names></name> <name><surname>Yau</surname> <given-names>T</given-names></name></person-group> <article-title>Cardiac Tumours: Diagnosis and Management</article-title> <source>Lancet Oncol</source> <year>2005</year><volume>6</volume><issue>4</issue><fpage>219</fpage><lpage>228</lpage><pub-id pub-id-type="doi">10.1016/S1470-2045(05)70093-0</pub-id></element-citation>
<mixed-citation>Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T. Cardiac Tumours: Diagnosis and Management. Lancet Oncol. 2005;6(4):219-28. doi: 10.1016/S1470-2045(05)70093-0.</mixed-citation></ref>
<ref id="B7"><label>7</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Bruce</surname> <given-names>CJ</given-names></name></person-group> <article-title>Cardiac Tumours: Diagnosis and Management</article-title> <source>Heart</source> <year>2011</year><volume>97</volume><issue>2</issue><fpage>151</fpage><lpage>160</lpage><pub-id pub-id-type="doi">10.1136/hrt.2009.186320</pub-id></element-citation>
<mixed-citation>Bruce CJ. Cardiac Tumours: Diagnosis and Management. Heart. 2011;97(2):151-60. doi: 10.1136/hrt.2009.186320.</mixed-citation></ref>
<ref id="B8"><label>8</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Biersmith</surname> <given-names>MA</given-names></name> <name><surname>Tong</surname> <given-names>MS</given-names></name> <name><surname>Guha</surname> <given-names>A</given-names></name> <name><surname>Simonetti</surname> <given-names>OP</given-names></name> <name><surname>Addison</surname> <given-names>D</given-names></name></person-group> <article-title>Multimodality Cardiac Imaging in the Era of Emerging Cancer Therapies</article-title> <source>J Am Heart Assoc</source> <year>2020</year><volume>9</volume><issue>2</issue><elocation-id>e013755</elocation-id><pub-id pub-id-type="doi">10.1161/JAHA.119.013755</pub-id></element-citation>
<mixed-citation>Biersmith MA, Tong MS, Guha A, Simonetti OP, Addison D. Multimodality Cardiac Imaging in the Era of Emerging Cancer Therapies. J Am Heart Assoc. 2020;9(2):e013755. doi: 10.1161/JAHA.119.013755.</mixed-citation></ref>
<ref id="B9"><label>9</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Zaragoza-Macias</surname> <given-names>E</given-names></name> <name><surname>Chen</surname> <given-names>MA</given-names></name> <name><surname>Gill</surname> <given-names>EA</given-names></name></person-group> <article-title>Real Time Three-Dimensional Echocardiography Evaluation of Intracardiac Masses</article-title> <source>Echocardiography</source> <year>2012</year><volume>29</volume><issue>2</issue><fpage>207</fpage><lpage>219</lpage><pub-id pub-id-type="doi">10.1111/j.1540-8175.2011.01627.x</pub-id></element-citation>
<mixed-citation>Zaragoza-Macias E, Chen MA, Gill EA. Real Time Three-Dimensional Echocardiography Evaluation of Intracardiac Masses. Echocardiography. 2012;29(2):207-19. doi: 10.1111/j.1540-8175.2011.01627.x.</mixed-citation></ref>
<ref id="B10"><label>10</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Motwani</surname> <given-names>M</given-names></name> <name><surname>Kidambi</surname> <given-names>A</given-names></name> <name><surname>Herzog</surname> <given-names>BA</given-names></name> <name><surname>Uddin</surname> <given-names>A</given-names></name> <name><surname>Greenwood</surname> <given-names>JP</given-names></name> <name><surname>Plein</surname> <given-names>S</given-names></name></person-group> <article-title>MR Imaging of Cardiac Tumors and Masses: A Review of Methods and Clinical Applications</article-title> <source>Radiology</source> <year>2013</year><volume>268</volume><issue>1</issue><fpage>26</fpage><lpage>43</lpage><pub-id pub-id-type="doi">10.1148/radiol.13121239</pub-id></element-citation>
<mixed-citation>Motwani M, Kidambi A, Herzog BA, Uddin A, Greenwood JP, Plein S. MR Imaging of Cardiac Tumors and Masses: A Review of Methods and Clinical Applications. Radiology. 2013;268(1):26-43. doi: 10.1148/radiol.13121239.</mixed-citation></ref>
<ref id="B11"><label>11</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Jeong</surname> <given-names>D</given-names></name> <name><surname>Patel</surname> <given-names>A</given-names></name> <name><surname>Francois</surname> <given-names>CJ</given-names></name> <name><surname>Gage</surname> <given-names>KL</given-names></name> <name><surname>Fradley</surname> <given-names>MG</given-names></name></person-group> <article-title>Cardiac Magnetic Resonance Imaging in Oncology</article-title> <source>Cancer Control</source> <year>2017</year><volume>24</volume><issue>2</issue><fpage>147</fpage><lpage>160</lpage><pub-id pub-id-type="doi">10.1177/107327481702400207</pub-id></element-citation>
<mixed-citation>Jeong D, Patel A, Francois CJ, Gage KL, Fradley MG. Cardiac Magnetic Resonance Imaging in Oncology. Cancer Control. 2017;24(2):147-60. doi: 10.1177/107327481702400207.</mixed-citation></ref>
<ref id="B12"><label>12</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Esposito</surname> <given-names>A</given-names></name> <name><surname>De Cobelli</surname> <given-names>F</given-names></name> <name><surname>Ironi</surname> <given-names>G</given-names></name> <name><surname>Marra</surname> <given-names>P</given-names></name> <name><surname>Canu</surname> <given-names>T</given-names></name> <name><surname>Mellone</surname> <given-names>R</given-names></name> <etal/></person-group> <article-title>CMR in the Assessment of Cardiac Masses: Primary Malignant Tumors</article-title> <source>JACC Cardiovasc Imaging</source> <year>2014</year><volume>7</volume><issue>10</issue><fpage>1057</fpage><lpage>1061</lpage><pub-id pub-id-type="doi">10.1016/j.jcmg.2014.08.002</pub-id></element-citation>
<mixed-citation>Esposito A, De Cobelli F, Ironi G, Marra P, Canu T, Mellone R, et al. CMR in the Assessment of Cardiac Masses: Primary Malignant Tumors. JACC Cardiovasc Imaging. 2014;7(10):1057-61. doi: 10.1016/j.jcmg.2014.08.002.</mixed-citation></ref>
</ref-list>
</back>
<sub-article article-type="translation" id="S1" xml:lang="pt">
<front-stub>
<article-id pub-id-type="doi">10.36660/abcimg.20250033</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Relato de Caso</subject>
</subj-group>
</article-categories>
<title-group>
<article-title>Turmoil de Sintomas: O Impacto Devastador de um Linfoma Cardíaco – Múltiplas Manifestações Clínicas e as Causas Letais de Acometimento Cardíaco</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0000-0001-7839-3965</contrib-id>
<name><surname>Carvalho</surname><given-names>Gustavo</given-names></name>
<role>Concepção e desenho da pesquisa</role>
<role>obtenção de dados</role>
<role>análise e interpretação dos dados</role>
<role>redação do manuscrito</role>
<role>revisão crítica do manuscrito quanto ao conteúdo intelectual importante</role>
<xref ref-type="aff" rid="aff4"><sup>1</sup></xref><xref ref-type="corresp" rid="c2"/></contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0009-0008-0807-4107</contrib-id>
<name><surname>Carvalho</surname><given-names>Maria Fernanda Miranda</given-names></name>
<role>obtenção de dados</role>
<role>análise e interpretação dos dados</role>
<role>redação do manuscrito</role>
<role>revisão crítica do manuscrito quanto ao conteúdo intelectual importante</role>
<role>revisão das referências</role>
<xref ref-type="aff" rid="aff4"><sup>1</sup></xref></contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0000-0002-7232-4552</contrib-id>
<name><surname>Magalhães</surname><given-names>Tiago</given-names></name>
<role>obtenção de dados</role>
<role>revisão crítica do manuscrito quanto ao conteúdo intelectual importante</role>
<role>imagens da ressonância magnética</role>
<xref ref-type="aff" rid="aff4"><sup>1</sup></xref><xref ref-type="aff" rid="aff5"><sup>2</sup></xref></contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0000-0001-9014-2720</contrib-id>
<name><surname>Varela</surname><given-names>Alexandre Manoel</given-names></name>
<role>análise e interpretação dos dados</role>
<role>revisão crítica do manuscrito quanto ao conteúdo intelectual importante</role>
<xref ref-type="aff" rid="aff6"><sup>3</sup></xref></contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0009-0003-4528-4174</contrib-id>
<name><surname>Staszko</surname><given-names>Kamila Fernanda</given-names></name>
<role>obtenção de dados</role>
<role>revisão crítica do manuscrito quanto ao conteúdo intelectual importante</role>
<role>imagens do ecocardiograma</role>
<xref ref-type="aff" rid="aff4"><sup>1</sup></xref></contrib>
<contrib contrib-type="author">
<contrib-id contrib-id-type="orcid">0000-0001-5246-0453</contrib-id>
<name><surname>Hayashida</surname><given-names>Mariana Rie</given-names></name>
<role>obtenção de dados</role>
<role>revisão crítica do manuscrito quanto ao conteúdo intelectual importante</role>
<role>análise da anatomia patológica</role>
<xref ref-type="aff" rid="aff4"><sup>1</sup></xref></contrib>
<aff id="aff4">
<label>1</label>
<addr-line>
<named-content content-type="city">Curitiba</named-content>
<named-content content-type="state">PR</named-content>
</addr-line>
<country country="BR">Brasil</country>
<institution content-type="original">Universidade Federal do Paraná, Curitiba, PR – Brasil</institution>
</aff>
<aff id="aff5">
<label>2</label>
<addr-line>
<named-content content-type="city">São Paulo</named-content>
<named-content content-type="state">SP</named-content>
</addr-line>
<country country="BR">Brasil</country>
<institution content-type="original">Hospital do Coração, São Paulo, SP – Brasil</institution>
</aff>
<aff id="aff6">
<label>3</label>
<addr-line>
<named-content content-type="city">Curitiba</named-content>
<named-content content-type="state">PR</named-content>
</addr-line>
<country country="BR">Brasil</country>
<institution content-type="original">Hospital Erasto Gaertner, Curitiba, PR – Brasil</institution>
</aff>
</contrib-group>
<author-notes>
<corresp id="c2"><label>Correspondência:</label> <bold>Gustavo Carvalho</bold> • CHC UFPR. Dionira M Klemtz, 201, casa 107. CEP: <postal-code>81320-390</postal-code>. Bairro Fazendinha, Curitiba, PR – Brasil E-mail: <email>gustavocarvalho1975@gmail.com</email></corresp>
<fn fn-type="conflict"><label>Potencial Conflito de Interesse</label>
<p>Declaro não haver conflito de interesses pertinentes.</p></fn>
<fn fn-type="edited-by"><label>Editor responsável pela revisão:</label> <p>Marcelo Tavares</p></fn>
</author-notes>
<abstract>
<title>Resumo</title>
<p>O linfoma cardíaco primário (LCP) é uma neoplasia rara e altamente letal, cuja apresentação clínica inespecífica dificulta o diagnóstico precoce. Este artigo relata o caso de um paciente idoso, previamente saudável, que apresentou tonturas e síncopes por quatro meses antes da descoberta de uma grande massa tumoral no coração. Exames de imagem, como ecocardiograma e ressonância magnética, foram cruciais para a identificação do tumor, e a biópsia confirmou tratar-se de um linfoma de células B com alta replicação. No entanto, diante da gravidade do quadro e da recusa da família em prosseguir com o tratamento, o paciente evoluiu para morte súbita duas semanas após o diagnóstico. Os tumores cardíacos primários (TCPs) são subdiagnosticados, com sintomas variados que podem incluir obstruções vasculares, arritmias e insuficiência cardíaca. Os LCPs representam apenas 1 a 2% das massas cardíacas primárias, sendo mais comuns em homens idosos. O diagnóstico é desafiador devido à ausência de sinais específicos, tornando exames de imagem e biópsias fundamentais para a confirmação da doença. O caso ilustra a complexidade do manejo dos LCPs, cuja evolução rápida limita as opções terapêuticas. A falta de sintomas específicos e a progressão silenciosa ressaltam a importância do diagnóstico precoce. A alta taxa de mortalidade reforça a necessidade de maior atenção médica para essa condição, que frequentemente leva a desfechos fatais antes da possibilidade de intervenção eficaz.</p>
</abstract>
<kwd-group xml:lang="pt">
<title>Palavras-chave</title>
<kwd>Neoplasias Cardíacas</kwd>
<kwd>Ecocardiografia</kwd>
<kwd>Arritmias Cardíacas</kwd>
<kwd>Biópsia</kwd>
</kwd-group>
</front-stub>
<body>
<sec sec-type="intro">
<title>Introdução</title>
<p>Os tumores cardíacos primários (TCPs) são entidades raras na prática clínica, podendo ser classificados como lesões benignas (mixomas) e malignas (sarcomas e linfomas).<sup><xref ref-type="bibr" rid="B1">1</xref>,<xref ref-type="bibr" rid="B2">2</xref></sup> Com o aumento da expectativa de vida da população, é esperado o incremento na incidência desses tumores, assim, cresce a necessidade de maior atenção aos seus possíveis sintomas, principalmente ao considerar que suas manifestações clínicas são diversas.<sup><xref ref-type="bibr" rid="B2">2</xref>,<xref ref-type="bibr" rid="B3">3</xref></sup></p>
<p>Tendo isso em vista, é importante ressaltar que os TCPs frequentemente são subdiagnosticados, podendo levar o paciente à óbito antes que uma abordagem terapêutica possa ser efetivamente implementada.<sup><xref ref-type="bibr" rid="B2">2</xref></sup> No caso a ser discorrido neste artigo, é retratado um linfoma miocárdico primário em um idoso, previamente hígido, que evoluiu para morte súbita duas semanas após o diagnóstico.</p>
<p>Com isso, o objetivo desse estudo é discutir as particularidades clínicas e diagnósticas dos TCPs.</p>
</sec>
<sec sec-type="cases">
<title>Relato de Caso</title>
<p>Homem, 78 anos de idade, previamente hígido, com queixa de tonturas e síncope com evolução de 4 meses. Chegou a ser atendido em outras unidades de pronto atendimento, porém houve piora progressiva de suas insatisfações.</p>
<p>Ao exame físico, nada digno de nota. No eletrocardiograma, ritmo sinusal e alteração difusa da repolarização ventricular. Radiografia de tórax demonstrando área cardíaca no limite superior da normalidade e velamento dos seios costofrênicos bilateralmente. Ao ecocardiograma transtorácico foi constatado grande massa tumoral infiltrada no septo interventricular e na parede ínfero-lateral do ventrículo direito, invadindo as cavidades atrial e ventricular direita, e a cavidade ventricular esquerda junto ao septo (<xref ref-type="fig" rid="f4">Figura 1</xref>).</p>
<fig id="f4">
<label>Figura 1</label>
<caption><title>Ecocardiograma transtorácico demonstrando massa tumoral infiltrada nas válvulas atrioventriculares, no miocárdio ventricular ao nível do septo, paredes anterior e inferior de ambos ventrículos. Além disso, o tumor ocupa cavidades atriais e ventricular direita (setas). Derrame pericárdico.</title></caption>
<graphic xlink:href="2675-312X-abcic-38-03-e20250033-gf01-pt.tif"/>
</fig>
<p>Diante disso, foi encaminhado para exame de ressonância magnética para melhor estudo da neoplasia, que corroborou os achados do ecocardiograma (<xref ref-type="fig" rid="f5">Figura 2</xref>). A biópsia endomiocárdica foi realizada guiada pelo ecocardiograma transtorácico e pela fluoroscopia, realizados simultaneamente na sala de Hemodinâmica. O estudo anatomopatológico e a análise imuno-histoquímica concluíram se tratar de um linfoma de células B invasivo com intensa replicação celular (<xref ref-type="fig" rid="f6">Figura 3</xref>).</p>
<fig id="f5">
<label>Figura 2</label>
<caption><title>Ressonância Magnética demonstrando a extensão do envolvimento cardíaco pelo linfoma (*) demonstrados nos eixos sagital (A) e transversal (B), além de derrame pericárdico e pleural bilateral.</title></caption>
<graphic xlink:href="2675-312X-abcic-38-03-e20250033-gf02-pt.tif"/>
</fig>
<fig id="f6">
<label>Figura 3</label>
<caption><title>Achados histopatológicos e de imuno-histoquímica demonstrando o linfoma de células B com intensa proliferação celular no kie67 (A), e os linfócitos B marcados no CD20 (B).</title></caption>
<graphic xlink:href="2675-312X-abcic-38-03-e20250033-gf03-pt.tif"/>
</fig>
<p>Após o esclarecimento detalhado do caso clínico complexo e grave para o próprio paciente e seus familiares, eles se recusaram implementação de qualquer tipo de tratamento e solicitaram alta para seu domicílio, onde permaneceu assintomático por cerca de mais duas semanas, quando apresentou morte súbita.</p>
</sec>
<sec sec-type="discussion">
<title>Discussão</title>
<p>Os TCPs, apesar de serem relatados desde o século XVI, são pouco frequentes na prática clínica, variando de <bold>0,0017% a 0,03%</bold> em estudos de necrópsia.<sup><xref ref-type="bibr" rid="B1">1</xref>-<xref ref-type="bibr" rid="B3">3</xref></sup> Podem se manifestar principalmente como lesões benignas, com uma taxa de incidência de 75%, ou como malignas, com 25%.<sup><xref ref-type="bibr" rid="B3">3</xref>-<xref ref-type="bibr" rid="B5">5</xref></sup></p>
<p>Os linfomas cardíacos primários (LCPs), por sua vez, são classificados como uma neoplasia maligna, representando cerca de 1 a 2% de todos os casos de massas cardíacas primárias.<sup><xref ref-type="bibr" rid="B2">2</xref>-<xref ref-type="bibr" rid="B5">5</xref></sup> Apesar de, em geral, os TCPs acometerem mais mulheres, os linfomas cardíacos primários são mais comuns em homens acima de 60 anos, fato bem representado pelo paciente descrito neste relato.<sup><xref ref-type="bibr" rid="B4">4</xref>-<xref ref-type="bibr" rid="B6">6</xref></sup></p>
<p>Os LCPs reproduzem quadros clínicos muitas vezes inespecíficos e multivariados. Os pacientes acometidos podem ser, inicialmente, desde assintomáticos e oligossintomáticos, que com sua evolução, apresentam sinais e sintomas diversos decorrente do acometimento direto e/ou indireto de múltiplos órgãos e sistemas, por mecanismos heterogêneos, simultâneos e diversificados, como os fenômenos vasculares obstrutivos recorrentes por trombos e/ou fragmentos tumorais (acidente vascular cerebral, embolias arteriais periféricas e viscerais e trombo embolia pulmonar).<sup><xref ref-type="bibr" rid="B4">4</xref>-<xref ref-type="bibr" rid="B7">7</xref></sup></p>
<p>O próprio tumor pode ocasionar uma obstrução fixa ou transitória do fluxo sanguíneo intracardíaco, a depender de suas dimensões e morfologia (intramural ou intracavitário pedunculado), e ainda, comprometendo o débito cardíaco.<sup><xref ref-type="bibr" rid="B2">2</xref>,<xref ref-type="bibr" rid="B4">4</xref></sup> Uma vez que há o acometimento do sistema de condução elétrico do miocárdio, as arritmias podem se fazer presentes, e inesperadamente fatais, como possivelmente ocorreu no caso aqui apresentado.<sup><xref ref-type="bibr" rid="B5">5</xref>-<xref ref-type="bibr" rid="B7">7</xref></sup></p>
<p>Tendo isso em vista, diante de casos de tumores cardíacos, é inegável que com a sintomatologia inespecífica, firmar um diagnóstico a tempo de se aplicar uma terapêutica efetiva é considerado um desafio para a equipe de assistência à saúde, assim como fora demonstrado no caso deste relato.<sup><xref ref-type="bibr" rid="B6">6</xref>,<xref ref-type="bibr" rid="B7">7</xref></sup> Outrossim, faz-se imprescindível ressaltar a importância dos exames de imagem como o ecocardiograma e a ressonância magnética cardíaca, no papel de detecção dessas massas tumorais que também auxiliam na realização das biópsias endomiocárdica, para que um diagnóstico histopatológico seja concretamente definido.<sup><xref ref-type="bibr" rid="B8">8</xref>-<xref ref-type="bibr" rid="B12">12</xref></sup></p>
</sec>
<sec sec-type="conclusions">
<title>Conclusão</title>
<p>O linfoma cardíaco primário (LCP) representa uma condição rara, mas de extrema gravidade, cuja apresentação clínica inespecífica e evolução silenciosa tornam seu diagnóstico um grande desafio. Como discutido neste relato, suas múltiplas manifestações podem mascarar a real extensão da doença, resultando em um prognóstico desalentador. O acometimento cardíaco pelo linfoma não apenas compromete a função do órgão, mas também expõe o paciente a um alto risco de eventos letais, como arritmias fatais e obstruções vasculares críticas.</p>
<p>Diante desse cenário, a importância de uma investigação precoce e criteriosa, utilizando exames de imagem, aspectos histopatológicos e imuno-histoquímicos para a definição diagnóstica é fundamental. No entanto, como evidenciado no caso descrito, mesmo com o diagnóstico estabelecido, as opções terapêuticas podem ser limitadas pela rápida progressão da doença e pelas decisões do próprio paciente.</p>
<p>Assim, o impacto devastador do linfoma cardíaco vai além das suas repercussões fisiológicas, estendendo-se à limitação terapêutica e ao desfecho abrupto, ressaltando a necessidade de maior atenção e entendimento médico-científico para futuras abordagens dessa entidade insólita e fatal.</p>
</sec>
</body>
<back>
<fn-group>
<fn fn-type="financial-disclosure" id="fn5"><label>Fontes de Financiamento</label>
<p>O presente estudo não teve fontes de financiamento externas.</p></fn>
<fn fn-type="other" id="fn6"><label>Vinculação Acadêmica</label>
<p>Não há vinculação deste estudo a programas de pós-graduação.</p></fn>
<fn fn-type="other" id="fn7"><label>Aprovação Ética e Consentimento Informado</label>
<p>Este estudo foi aprovado pelo Comitê de Ética do Hospital De Clínicas Da Universidade Federal Do Paraná sob o número de protocolo 90118225.4.0000.0096. Todos os procedimentos envolvidos nesse estudo estão de acordo com a Declaração de Helsinki de 1975, atualizada em 2013. Houve dispensa do Termo de Consentimento Livre e Esclarecido.</p></fn>
<fn fn-type="other" id="fn8"><label>Uso de Inteligência Artificial</label>
<p>Os autores não utilizaram ferramentas de inteligência artificial no desenvolvimento deste trabalho.</p></fn>
</fn-group>
<sec sec-type="data-availability" specific-use="data-in-article">
<label>Disponibilidade de Dados</label>
<p>Os conteúdos subjacentes ao texto da pesquisa estão contidos no manuscrito.</p>
</sec>
</back>
</sub-article>
</article>