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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 70-year-old woman was referred to the emergency department due to mild effort dyspnea&#44; weight loss and night sweats&#46; Transthoracic and transesophageal echocardiography revealed dilated left atrium with an extensive multilobulated mass infiltrating the left atrial posterolateral wall&#46; It prolapsed through the mitral valve during diastole&#44; resulting in elevated mean and peak pressure gradients &#40;8 mmHg and 25 mmHg&#44; respectively &#91;Panels A-C&#93;&#41;&#46; Coronary angiography revealed a highly vascularized mass &#40;Panel D&#44; <a class="elsevierStyleCrossRef" href="#sec0010">Video 1</a>&#41;&#46; Cardiac magnetic resonance &#40;CMR&#41; evidenced the full extension of the mass &#8211; measuring 10 cm &#8211; in relation to the left atrial posterolateral wall&#46; It extended to both inferior pulmonary veins and revealed tissue characteristics in T1- &#40;isointense&#41; and T2-weighted &#40;hyperintense&#41; images&#46; No contrast uptake was found during first-pass perfusion&#44; but progressive and heterogeneous uptake was observed in the early and late gadolinium enhancement &#40;LGE&#41; images&#44; with a low signal intensity central area &#40;Panels E-G&#44; <a class="elsevierStyleCrossRef" href="#sec0010">Video 2 and 3</a>&#41;&#46; Computed tomography &#40;CT&#41; staging was negative for metastases&#46; The patient was referred for surgery&#44; but only partial resection was possible &#40;Panel H&#41;&#46; Histopathology revealed a malignant neoplasm&#44; with high cellularity&#44; which was predominantly undifferentiated and pleomorphic&#44; with fusiform and epithelioid cells&#59; necrosis areas and a high mitotic index &#40;Panel I&#41;&#59; vimentin &#40;Panel J&#41; and nuclear multifocal MDM2 expression &#40;Panel K&#41;&#46; These findings are consistent with intimal sarcoma&#46; The patient was not considered to be a candidate for chemotherapy due to the tumor characteristics&#46; One month later&#44; congestive symptoms relapsed&#46; A CT scan confirmed mass growth and pulmonary vein invasion &#40;Panel L&#41;&#46; The patient died within a month &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">Primary cardiac tumors are rare &#40;incidence &#60;0&#46;03&#37;&#41; and only one quarter is malignant&#46; Intimal sarcomas are mesenchymal tumors and are more commonly encountered in great vessels&#44; so the heart is rarely involved&#46; To our knowledge&#44; only eight cases have been reported to date&#46; These tumors are locally aggressive and proliferate rapidly&#46; Although surgical excision with tumor-free margins is the main treatment&#44; complete surgical excision may often not be possible&#46; The overall prognosis is poor&#44; with a median survival of 3 to 12 months&#46; The effectiveness of chemotherapy and radiotherapy is the subject of debate&#46; In most cases&#44; the cause of death is local tumor enlargement and recurrence&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Echocardiography is the screening method of choice for cardiac tumors &#40;sensitivity 93&#46;3-96&#37;&#41; and is useful for differentiating malignant neoplasms from benign ones&#46; However&#44; due to a limited field of view&#44; the tumor burden may remain underdiagnosed&#46; CMR&#44; which offers multiplanar imaging without restrictions on the field of view&#44; enables the tumor to be accurately located&#44; the extent of involvement to be assessed and the functional impact of the lesion and tissue characterization to be evaluated&#46; In this case&#44; pulmonary vein involvement&#44; broad-based attachment&#44; size greater than 5 cm&#44; ill-defined margins&#44; heterogeneous signal on T1-and T2-weighted images and heterogeneous LGE all led to suspicion of malignancy&#46; However&#44; CMR features are non-specific&#44; with intermediate T1 and high T2 signal intensities and varying amounts of first-pass uptake and LGE&#46; An accurate diagnosis could only be made by histopathology&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">In this rare case of mitral valve obstruction&#44; multimodality imaging was crucial to guide diagnosis and treatment&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0025" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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Image in Cardiology
Intimal sarcoma of the left atrium – A rare form of mitral valve obstruction
Sarcoma da íntima da aurícula esquerda – uma forma rara de obstrução valvular mitral
Glória Abreua,
Corresponding author
gloriappabreu@gmail.com

Corresponding author.
, Alberto Salgadoa, Nuno Bettencourta,b, Nuno Saloméa, João Ferreirac, Susana Guimarãesd
a Serviço de Cardiologia, Hospital de Braga, Braga, Portugal
b Faculdade de Medicina da Universidade do Porto, Porto, Portugal
c Serviço de Cirurgia Cardiotorácica, Centro Hospital de São João, Porto, Portugal
d Serviço de Anatomia Patológica, Centro Hospitalar de São João, Porto, Portugal
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Article information
ISSN: 21742049
Original language: English
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Revista Portuguesa de Cardiologia (English edition)
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