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Inflammatory myofibroblastic tumor of the heart: A brief overview
Tumor miofibroblástico inflamatório do coração: breve visão global
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"apellidos" => "Blanco-Herrera" ] 3 => array:2 [ "nombre" => "Tomás" "apellidos" => "Heredia-Cambra" ] 4 => array:2 [ "nombre" => "Manuel" "apellidos" => "Pérez-Guillén" ] 5 => array:2 [ "nombre" => "Vicent" "apellidos" => "Martínez-Cózar" ] 6 => array:2 [ "nombre" => "Empar" "apellidos" => "Mayordomo-Aranda" ] 7 => array:2 [ "nombre" => "Fernando" "apellidos" => "Hornero-Sos" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S087025512200525X?idApp=UINPBA00004E" "url" => "/08702551/0000004200000002/v1_202302081302/S087025512200525X/v1_202302081302/en/main.assets" ] ] "en" => array:12 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Editorial comment</span>" "titulo" => "Inflammatory myofibroblastic tumor of the heart: A brief overview" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "171" "paginaFinal" => "172" ] ] "autores" => array:1 [ 0 => array:3 [ "autoresLista" => "Rosa Henriques de Gouveia" "autores" => array:1 [ 0 => array:4 [ "nombre" => "Rosa" "apellidos" => "Henriques de Gouveia" "email" => array:1 [ 0 => "rhgouveia@mail.telepac.pt" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Histology & Pathology (Medicine), Faculty of Life Sciences, University of Madeira (UMa), Funchal, Madeira, Portugal" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "LANA – Clinical and Anatomical Pathology Laboratory, Funchal, Madeira, Portugal" "etiqueta" => "b" "identificador" => "aff0010" ] ] ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Tumor miofibroblástico inflamatório do coração: breve visão global" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Inflammatory myofibroblastic tumor (IMT) of the heart, also known as inflammatory pseudotumor among other designations, was first reported by Gonzalez-Crussi et al. in 1975.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a> It is rare (<5% of primary heart tumors),<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">2</span></a> with 62 cases reported in the international literature by 2021.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a> It can affect any age group, but favors young persons.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">2</span></a> Although found in multiple cardiac locations, the atria (particularly the right) are its preferred sites.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">2</span></a> Location and size affect clinical presentation and outcome, which range from asymptomatic and incidental, to constitutional symptoms such as fever and weight loss, to cardiological signs and symptoms (including chest pain, ischemia, valve occlusion or dysfunction, arrhythmia or syncope) and even sudden death.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">2–4</span></a> Imaging techniques are of paramount importance for diagnostic and follow-up workflow.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">2–4</span></a> Surgery is the usual therapeutic approach.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">2–4</span></a> Macroscopically, it is often pedunculated and endocardium-based.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">2</span></a> Microscopically, it is composed of spindle cells (fibroblasts and myofibroblasts) showing little or no atypia as well as no or very few mitoses (≤1 per 10 high-power fields), and inflammatory cells (mostly lympho-histio-plasmocytic), in a myxoid stroma.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a> Specific stains are used to characterize the spindle cells (Masson trichrome) and the stroma (periodic acid-Schiff and Alcian blue for mucinous material and elastic van Gieson to exclude elastic fibers).<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">2–4</span></a> Immunohistochemistry can further confirm the diagnosis (vimentin, smooth muscle actin, desmin, calponin and anaplastic lymphoma kinase I [ALK-I] positivity for the spindle cells). The inflammatory cells may also be marked. If ALK-I is negative (in around 50% of IMTs, apparently with worse prognosis), genetic tests may be performed, searching for 2p23 locus clonal ALK gene rearrangements, ALK gene fusion with proto-oncogenes and ALK overexpression, among others.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">2–4</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">The differential diagnosis is particularly with benign tumors like cardiac fibroma or myxoma, or with malignant neoplasms like sarcomas.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">4</span></a> IMT has an indolent growth, due to its intermediate biological nature.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">2–4</span></a> Mortality has been estimated at 24.6%, with metastasis in 2% and recurrence in 25%.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a> The underlying factors that lead to the development of IMT are unknown, although multiple theories have been proposed, including fibroblastic-inflammatory reaction to infection, trauma or iatrogenic aggression; autoimmune disease; or neoplastic chromosomal abnormalities.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">2–4</span></a> Apart from incomplete surgical resection, the ongoing pathological stimuli proposed by these etiological theories could explain potential IMT recurrence, the appearance of multiple synchronous or metachronous heart IMTs (in the same or different intracardiac locations) and even the malignant sarcomatous transformation of an IMT.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Regarding the case reported by Dominguez-Massa et al. in this issue of the <span class="elsevierStyleItalic">Journal</span>,<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">5</span></a> differentiation from an IMT to an intimal sarcoma in the right atrium, three years after surgery for an IMT in the left atrium, cannot be confirmed. It is known that immunohistochemical positivity for murine double minute 2 (MDM2) with or without fluorescence in-situ hybridization confirmation of amplification of the proto-oncogene <span class="elsevierStyleItalic">MDM2</span> – relevant in the diagnosis of intimal sarcoma – may also occur in IMT,<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">6</span></a> requiring accurate study in order to find the associated molecular aberration said to be typical of intimal sarcoma.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">7</span></a> This brief overview shows how complex IMT diagnosis can be.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0020" class="elsevierStylePara elsevierViewall">The author has no conflicts of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0040" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Unusual intracardiac tumor in a child. 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| 2023 Dezembro | 41 | 23 | 64 |
| 2023 Novembro | 43 | 40 | 83 |
| 2023 Outubro | 35 | 16 | 51 |
| 2023 Setembro | 37 | 29 | 66 |
| 2023 Agosto | 34 | 15 | 49 |
| 2023 Julho | 36 | 6 | 42 |
| 2023 Junho | 24 | 15 | 39 |
| 2023 Maio | 39 | 23 | 62 |
| 2023 Abril | 26 | 16 | 42 |
| 2023 Maro | 33 | 22 | 55 |
| 2023 Fevereiro | 72 | 77 | 149 |
| 2023 Janeiro | 33 | 18 | 51 |
| 2022 Dezembro | 19 | 32 | 51 |
Mostrar tudo
