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no evidence of invasion of the thoracic wall was found&#46;</p><elsevierMultimedia ident="fig0001"></elsevierMultimedia><p id="para0004" class="elsevierStylePara elsevierViewall">Surgical specimen histology revealed a spindle cell tumor&#44; with infiltration of bronchial structures &#40;<a class="elsevierStyleCrossRef" href="#fig0001">Fig&#46; 1</a>B&#41;&#44; and atypical neoplastic cells&#44; with frequent mitotic figures &#40;<a class="elsevierStyleCrossRef" href="#fig0001">Fig&#46; 1</a>C&#41;&#46; On immunohistochemical study&#44; tumoral cells expressed vimentin and B-cell lymphoma 2 &#40;BCL2&#41;&#46; There was loss of tri-methylation of lysine 27 on histone H3 protein &#40;H3K27me3&#41; expression in the tumoral cells&#46; Pancytokeratins &#40;AE1-AE3&#41;&#44; S100 protein&#44; desmin&#44; cluster of differentiation 34 &#40;CD34&#41; and Melan A were negative&#46; There was no translocation t&#40;x&#44;18&#41;&#46; MPNST was the suggested diagnosis&#46;</p><p id="para0005" class="elsevierStylePara elsevierViewall">After 3 months&#44; the patient started chemotherapy &#40;doxorubicin&#41;&#46; At this time&#44; he already presented metastatic lesions on the pectoralis major&#46; He died after 3 years&#44; with a hilar mass with invasion of the mediastinum&#44; which caused complete occlusion of the left main bronchus &#40;LMB&#41;&#46; In his last two years&#44; the patient was submitted to at least 5 rigid bronchoscopies&#44; for mechanical release&#44; and one prosthesis was placed at the LMB one year before he died&#46;</p><p id="para0006" class="elsevierStylePara elsevierViewall">Immunohistochemical and molecular studies are essential to diagnose of MPNSTs&#46;<a class="elsevierStyleCrossRef" href="#bib0002"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">&#44;</span><a class="elsevierStyleCrossRef" href="#bib0003"><span class="elsevierStyleSup">3</span></a></p><p id="para0007" class="elsevierStylePara elsevierViewall">MPNSTs are highly invasive&#44; with a low survival rate&#44; but surgery significantly improves disease-free survival&#46;<a class="elsevierStyleCrossRef" href="#bib0004"><span class="elsevierStyleSup">4</span></a> Chemotherapy is most used in unresectable or metastatic malignancies&#44; like in our patient&#44; but there are no formal treatment or palliative care guidelines&#46;<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&#46; Therefore&#44; every case is a challenge&#46; Despite being rare&#44; MPNSTs must be kept in mind&#46;</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&#46;</p></span></span>"
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A rare case of pulmonary malignant peripheral nerve sheath tumor
Rita Costaa,
Corresponding author
rita2ac@hotmail.com

Corresponding author at: Centro Hospitalar Universitário são João, Porto, Portugal, Alameda Prof. Hernâni Monteiro, 4200-319, Porto, Portugal.
, José Maximoa, Pedro Fernandesa, Susana Guimarãesb, Roberto Silvab, Adriana Magalhãesc
a Department of Cardiothoracic Surgery, Centro Hospitalar Universitário são João, Portugal
b Department of Pathology, Centro Hospitalar Universitário de São João, Porto, Portugal
c Department of Pulmology, Centro Hospitalar Universitário são João, Portugal
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          "en" => "<p id="spara001" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; PET scan image showing high accumulation of FDG in the pulmonary mass&#46; &#40;B&#41; Spindle cell tumor with infiltration of bronchial structures &#40;HE 200X&#41;&#46; Magnification bar&#160;&#61;&#160;200&#160;&#181;m&#46;&#40;C&#41; Atypical neoplastic cells with frequent mitotic figures &#40;HE 400x&#41;&#46; Magnification bar&#160;&#61;&#160;100&#160;&#181;m&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="para0001" class="elsevierStylePara elsevierViewall">Malignant peripheral nerve sheath tumors &#40;MPNSTs&#41; are extremely rare tumors &#40;0&#44;001&#37; incidence in the general population&#44; less than 10&#37; of all soft tissue sarcomas&#41; and&#44; in adulthood&#44; they are associated with neurofibromatosis type 1&#46;<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&#46;<a class="elsevierStyleCrossRef" href="#bib0002"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">&#44;</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&#44; with no relevant past medical history&#44; went to the emergency department complaining of chest discomfort over several days&#46; The patient had no family history of neurofibromatosis or caf&#233;-au-lait spots on physical examination&#46; The electrocardiogram and cardiac markers were normal&#44; but the x-ray showed a left hypotransparency&#46; The chest computed tomography &#40;CT&#41; presented a homogeneous spindle-shaped mass &#40;100&#160;&#215;&#160;70&#160;mm&#41;&#44; with no signs of invasion of mediastinal structures or lymphadenopathies&#46;</p><p id="para0003" class="elsevierStylePara elsevierViewall">The positron emission tomography &#40;PET&#41; revealed a high accumulation of fluorodeoxyglucose &#40;FDG&#41; in the mass &#40;<a class="elsevierStyleCrossRef" href="#fig0001">Fig&#46; 1</a>A&#41;&#46; Bronchofibroscopy did not show endobronchial lesions&#46; Two transbronchial lung biopsies were performed&#58; the first one had a small amount of material but revealed a probably neuroendocrine tumor&#59; the second biopsy showed only necrosis&#46; The patient underwent an upper left lobectomy and systematic lymph node dissection by thoracotomy&#59; no evidence of invasion of the thoracic wall was found&#46;</p><elsevierMultimedia ident="fig0001"></elsevierMultimedia><p id="para0004" class="elsevierStylePara elsevierViewall">Surgical specimen histology revealed a spindle cell tumor&#44; with infiltration of bronchial structures &#40;<a class="elsevierStyleCrossRef" href="#fig0001">Fig&#46; 1</a>B&#41;&#44; and atypical neoplastic cells&#44; with frequent mitotic figures &#40;<a class="elsevierStyleCrossRef" href="#fig0001">Fig&#46; 1</a>C&#41;&#46; On immunohistochemical study&#44; tumoral cells expressed vimentin and B-cell lymphoma 2 &#40;BCL2&#41;&#46; There was loss of tri-methylation of lysine 27 on histone H3 protein &#40;H3K27me3&#41; expression in the tumoral cells&#46; Pancytokeratins &#40;AE1-AE3&#41;&#44; S100 protein&#44; desmin&#44; cluster of differentiation 34 &#40;CD34&#41; and Melan A were negative&#46; There was no translocation t&#40;x&#44;18&#41;&#46; MPNST was the suggested diagnosis&#46;</p><p id="para0005" class="elsevierStylePara elsevierViewall">After 3 months&#44; the patient started chemotherapy &#40;doxorubicin&#41;&#46; At this time&#44; he already presented metastatic lesions on the pectoralis major&#46; He died after 3 years&#44; with a hilar mass with invasion of the mediastinum&#44; which caused complete occlusion of the left main bronchus &#40;LMB&#41;&#46; In his last two years&#44; the patient was submitted to at least 5 rigid bronchoscopies&#44; for mechanical release&#44; and one prosthesis was placed at the LMB one year before he died&#46;</p><p id="para0006" class="elsevierStylePara elsevierViewall">Immunohistochemical and molecular studies are essential to diagnose of MPNSTs&#46;<a class="elsevierStyleCrossRef" href="#bib0002"><span class="elsevierStyleSup">2</span></a><span class="elsevierStyleSup">&#44;</span><a class="elsevierStyleCrossRef" href="#bib0003"><span class="elsevierStyleSup">3</span></a></p><p id="para0007" class="elsevierStylePara elsevierViewall">MPNSTs are highly invasive&#44; with a low survival rate&#44; but surgery significantly improves disease-free survival&#46;<a class="elsevierStyleCrossRef" href="#bib0004"><span class="elsevierStyleSup">4</span></a> Chemotherapy is most used in unresectable or metastatic malignancies&#44; like in our patient&#44; but there are no formal treatment or palliative care guidelines&#46;<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&#46; Therefore&#44; every case is a challenge&#46; Despite being rare&#44; MPNSTs must be kept in mind&#46;</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&#46;</p></span></span>"
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Pulmonology

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