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A rare case of pulmonary malignant peripheral nerve sheath tumor
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Silva, L. Ferreira" "autores" => array:2 [ 0 => array:2 [ "nombre" => "J.M." "apellidos" => "Silva" ] 1 => array:2 [ "nombre" => "L." "apellidos" => "Ferreira" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2531043723001204?idApp=UINPBA00004E" "url" => "/25310437/0000002900000006/v3_202311162326/S2531043723001204/v3_202311162326/en/main.assets" ] "en" => array:15 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Photo</span>" "titulo" => "A rare case of pulmonary malignant peripheral nerve sheath tumor" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "574" "paginaFinal" => "575" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Rita Costa, José Maximo, Pedro Fernandes, Susana Guimarães, Roberto Silva, Adriana Magalhães" "autores" => array:6 [ 0 => array:4 [ "nombre" => "Rita" "apellidos" => "Costa" "email" => array:1 [ 0 => "rita2ac@hotmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0001" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0001" ] ] ] 1 => array:3 [ "nombre" => "José" "apellidos" => "Maximo" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0001" ] ] ] 2 => array:3 [ "nombre" => "Pedro" "apellidos" => "Fernandes" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0001" ] ] ] 3 => array:3 [ "nombre" => "Susana" "apellidos" => "Guimarães" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0002" ] ] ] 4 => array:3 [ "nombre" => "Roberto" "apellidos" => "Silva" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0002" ] ] ] 5 => array:3 [ "nombre" => "Adriana" "apellidos" => "Magalhães" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0003" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Department of Cardiothoracic Surgery, Centro Hospitalar Universitário são João, Portugal" "etiqueta" => "a" "identificador" => "aff0001" ] 1 => array:3 [ "entidad" => "Department of Pathology, Centro Hospitalar Universitário de São João, Porto, Portugal" "etiqueta" => "b" "identificador" => "aff0002" ] 2 => array:3 [ "entidad" => "Department of Pulmology, Centro Hospitalar Universitário são João, Portugal" "etiqueta" => "c" "identificador" => "aff0003" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0001" "etiqueta" => "⁎" "correspondencia" => "Corresponding author at: Centro Hospitalar Universitário são João, Porto, Portugal, Alameda Prof. Hernâni Monteiro, 4200-319, Porto, Portugal." ] ] ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0001" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 720 "Ancho" => 2918 "Tamanyo" => 426806 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "alt0001" "detalle" => "Fig " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spara001" class="elsevierStyleSimplePara elsevierViewall">(A) PET scan image showing high accumulation of FDG in the pulmonary mass. (B) Spindle cell tumor with infiltration of bronchial structures (HE 200X). Magnification bar = 200 µm.(C) Atypical neoplastic cells with frequent mitotic figures (HE 400x). Magnification bar = 100 µm.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="para0001" class="elsevierStylePara elsevierViewall">Malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare tumors (0,001% incidence in the general population, less than 10% of all soft tissue sarcomas) and, in adulthood, they are associated with neurofibromatosis type 1.<a class="elsevierStyleCrossRef" href="#bib0001"><span class="elsevierStyleSup">1</span></a> Primary pulmonary MPNSTs can mimic lung cancer but there are few literature case reports.<a class="elsevierStyleCrossRef" href="#bib0002"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">,</span><a class="elsevierStyleCrossRef" href="#bib0003"><span class="elsevierStyleSup">3</span></a></p><p id="para0002" class="elsevierStylePara elsevierViewall">A 53-year-old non-smoker male, with no relevant past medical history, went to the emergency department complaining of chest discomfort over several days. The patient had no family history of neurofibromatosis or café-au-lait spots on physical examination. The electrocardiogram and cardiac markers were normal, but the x-ray showed a left hypotransparency. The chest computed tomography (CT) presented a homogeneous spindle-shaped mass (100 × 70 mm), with no signs of invasion of mediastinal structures or lymphadenopathies.</p><p id="para0003" class="elsevierStylePara elsevierViewall">The positron emission tomography (PET) revealed a high accumulation of fluorodeoxyglucose (FDG) in the mass (<a class="elsevierStyleCrossRef" href="#fig0001">Fig. 1</a>A). Bronchofibroscopy did not show endobronchial lesions. Two transbronchial lung biopsies were performed: the first one had a small amount of material but revealed a probably neuroendocrine tumor; the second biopsy showed only necrosis. The patient underwent an upper left lobectomy and systematic lymph node dissection by thoracotomy; no evidence of invasion of the thoracic wall was found.</p><elsevierMultimedia ident="fig0001"></elsevierMultimedia><p id="para0004" class="elsevierStylePara elsevierViewall">Surgical specimen histology revealed a spindle cell tumor, with infiltration of bronchial structures (<a class="elsevierStyleCrossRef" href="#fig0001">Fig. 1</a>B), and atypical neoplastic cells, with frequent mitotic figures (<a class="elsevierStyleCrossRef" href="#fig0001">Fig. 1</a>C). On immunohistochemical study, tumoral cells expressed vimentin and B-cell lymphoma 2 (BCL2). There was loss of tri-methylation of lysine 27 on histone H3 protein (H3K27me3) expression in the tumoral cells. Pancytokeratins (AE1-AE3), S100 protein, desmin, cluster of differentiation 34 (CD34) and Melan A were negative. There was no translocation t(x,18). MPNST was the suggested diagnosis.</p><p id="para0005" class="elsevierStylePara elsevierViewall">After 3 months, the patient started chemotherapy (doxorubicin). At this time, he already presented metastatic lesions on the pectoralis major. He died after 3 years, with a hilar mass with invasion of the mediastinum, which caused complete occlusion of the left main bronchus (LMB). In his last two years, the patient was submitted to at least 5 rigid bronchoscopies, for mechanical release, and one prosthesis was placed at the LMB one year before he died.</p><p id="para0006" class="elsevierStylePara elsevierViewall">Immunohistochemical and molecular studies are essential to diagnose of MPNSTs.<a class="elsevierStyleCrossRef" href="#bib0002"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">,</span><a class="elsevierStyleCrossRef" href="#bib0003"><span class="elsevierStyleSup">3</span></a></p><p id="para0007" class="elsevierStylePara elsevierViewall">MPNSTs are highly invasive, with a low survival rate, but surgery significantly improves disease-free survival.<a class="elsevierStyleCrossRef" href="#bib0004"><span class="elsevierStyleSup">4</span></a> Chemotherapy is most used in unresectable or metastatic malignancies, like in our patient, but there are no formal treatment or palliative care guidelines.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">5</span></a> Rare tumors are associated with difficulties in diagnosis and evidence-based treatments are lacking. Therefore, every case is a challenge. Despite being rare, MPNSTs must be kept in mind.</p><span id="sec0001" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="cesectitle0002">Ethical considerations</span><p id="para0008" class="elsevierStylePara elsevierViewall">Written informed consent was obtained from the patient guardian for publication of the article.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0001" "titulo" => "Ethical considerations" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2022-11-10" "fechaAceptado" => "2023-01-23" "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "fig0001" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 720 "Ancho" => 2918 "Tamanyo" => 426806 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "alt0001" "detalle" => "Fig " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spara001" class="elsevierStyleSimplePara elsevierViewall">(A) PET scan image showing high accumulation of FDG in the pulmonary mass. (B) Spindle cell tumor with infiltration of bronchial structures (HE 200X). Magnification bar = 200 µm.(C) Atypical neoplastic cells with frequent mitotic figures (HE 400x). Magnification bar = 100 µm.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "cebibsec1" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0001" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Primary malignantperipheral nerve sheath tumor of the lung in a young child withoutneurofibromatosis type 1" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "S.A. Muwakkit" 1 => "C. Rodriguez-Galindo" 2 => "A.I. El Samra" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1002/pbc.20787" "Revista" => array:6 [ "tituloSerie" => "Pediatr Blood Cancer" "fecha" => "2006" "volumen" => "47" "paginaInicial" => "636" "paginaFinal" => "638" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16544294" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0002" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Primary intrapulmonary malignant peripheral nerve sheath tumor mimicking lung cancer" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "E. La Mantia" 1 => "R. Franco" 2 => "M. Cantile" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.3978/j.issn.2072-1439.2013.07.02" "Revista" => array:7 [ "tituloSerie" => "J Thorac Dis" "fecha" => "2013" "volumen" => "5" "numero" => "4" "paginaInicial" => "E155" "paginaFinal" => "E157" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23991328" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0003" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Primary intrapulmonary malignant peripheral nerve sheath tumor: a rare case" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "L.A. Maane" 1 => "A.A. Bouzidi" 2 => "M. Damou" 3 => "N. 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Mahendra" 3 => "V Chander" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.ijscr.2019.10.004" "Revista" => array:6 [ "tituloSerie" => "Int J Surg Case Rep" "fecha" => "2019" "volumen" => "64" "paginaInicial" => "161" "paginaFinal" => "164" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/31670144" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/25310437/0000002900000006/v3_202311162326/S2531043723000132/v3_202311162326/en/main.assets" "Apartado" => array:4 [ "identificador" => "88777" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Images" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/25310437/0000002900000006/v3_202311162326/S2531043723000132/v3_202311162326/en/main.pdf?idApp=UINPBA00004E&text.app=https://journalpulmonology.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2531043723000132?idApp=UINPBA00004E" ]
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