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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We describe a case of a 43-year-old man who was referred to the Dermatology Service&#44; reporting the progressive appearance of painful suppurative nodules in the right lower limb for six months&#46; The lesions started after penetrating trauma to the foot&#46; He had hardened nodules&#44; some of them ulcerated&#44; on the lateral aspect of the right lower limb&#44; in an ascending distribution &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Histopathology showed a malignant neoplasm infiltrating the skin and subcutaneous tissue&#44; epithelioid cells with granulomatous morphology and an area of necrosis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Cultures for bacteria&#44; fungi&#44; and mycobacteria showed no growth&#46; Immunohistochemistry showed reactivity for INI-1 and lysozyme&#46; There was focal reaction with CD68 and no reaction for EMA&#44; CD34&#44; desmin and S100&#44; which is compatible with epithelioid sarcoma &#40;ES&#41;&#46; Staging showed ipsilateral inguinal lymph node enlargement measuring 3&#46;1&#215;2&#46;0&#8239;cm&#46; He underwent chemotherapy with doxorubicin with control of the appearance of new lesions and healing of old lesions&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">ES is a rare subtype of soft tissue sarcoma with a high potential for local recurrence and metastases&#46; It preferentially affects young male adults and about a quarter of cases are associated with previous trauma&#44; such as the present one&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> It is subdivided into proximal type&#44; with a bad prognosis&#44; affecting the trunk&#44; axilla&#44; and perineum&#44; usually presenting as infiltrating masses in the subcutaneous tissue&#44; 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desmin&#44; CD68&#44; and CD31&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> CD68 positivity is uncommon&#44; but focal reaction in macrophages in ES associated with intense inflammatory infiltrate&#44; as in the present case&#44; has been reported&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Such a finding is mostly seen in granulomatous processes such as rheumatoid nodules and granuloma annulare&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> A common characteristic of ES&#44; present in more than 90&#37; of cases&#44; is the loss of expression of INI-1&#44; the tumor suppressor gene expressed in normal nucleated cells&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a> A minority of cases of ES&#44; as in the reported study&#44; maintains normal INI-1 expression and may be related to a more aggressive biological behavior&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">The differential diagnosis includes nodules due to infectious disease&#44; 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contributions</span><p id="par0030" class="elsevierStylePara elsevierViewall">Vivian Spanemberg Macuglia&#58; Approval of the final version of the manuscript&#59; design and planning of the study&#59; drafting and editing of the manuscript&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Juliano Peruzzo&#58; Approval of the final version of the manuscript&#59; design and planning of the study&#59; drafting and editing of the manuscript&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Ariane Bastos Geller&#58; Approval of the final version of the manuscript&#59; design and planning of the study&#59; drafting and editing of the manuscript&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Renan Rangel Bonamigo&#58; Approval of the final version of the manuscript&#59; design and planning of the study&#59; drafting and editing of the manuscript&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0050" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span></span>"
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Letter - Clinical
Skin nodules of distal-type epithelioid sarcoma
Vivian Spanemberg Macugliaa,
Autor para correspondência
, Juliano Peruzzoa, Ariane Bastos Gellera, Renan Rangel Bonamigoa,b
a Department of Dermatology, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil
b Faculty of Medicine ‒ Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We describe a case of a 43-year-old man who was referred to the Dermatology Service&#44; reporting the progressive appearance of painful suppurative nodules in the right lower limb for six months&#46; The lesions started after penetrating trauma to the foot&#46; He had hardened nodules&#44; some of them ulcerated&#44; on the lateral aspect of the right lower limb&#44; in an ascending distribution &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Histopathology showed a malignant neoplasm infiltrating the skin and subcutaneous tissue&#44; epithelioid cells with granulomatous morphology and an area of necrosis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Cultures for bacteria&#44; fungi&#44; and mycobacteria showed no growth&#46; Immunohistochemistry showed reactivity for INI-1 and lysozyme&#46; There was focal reaction with CD68 and no reaction for EMA&#44; CD34&#44; desmin and S100&#44; which is compatible with epithelioid sarcoma &#40;ES&#41;&#46; Staging showed ipsilateral inguinal lymph node enlargement measuring 3&#46;1&#215;2&#46;0&#8239;cm&#46; He underwent chemotherapy with doxorubicin with control of the appearance of new lesions and healing of old lesions&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">ES is a rare subtype of soft tissue sarcoma with a high potential for local recurrence and metastases&#46; It preferentially affects young male adults and about a quarter of cases are associated with previous trauma&#44; such as the present one&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> It is subdivided into proximal type&#44; with a bad prognosis&#44; affecting the trunk&#44; axilla&#44; and perineum&#44; usually presenting as infiltrating masses in the subcutaneous tissue&#44; <a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> and distal type &#40;classic&#41;&#44; usually occurring in the extremities as firm&#44; painless&#44; slow-growing nodules&#46; Microscopically&#44; the classic form is composed of epithelioid cells with abundant eosinophilic cytoplasm in a fascicular arrangement&#44; surrounding a central necrotic area&#44; producing a granulomatous pattern &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> In the proximal variant&#44; on the other hand&#44; the cells have a rhabdoid morphology and increased atypia&#46; There may be &#8220;signet-ring&#8221; vacuolation&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> ES is positive for both epithelial and mesenchymal markers&#46; CD34 and epithelial membrane antigen are often positive&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Typical negative markers include S100&#44; desmin&#44; CD68&#44; and CD31&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> CD68 positivity is uncommon&#44; but focal reaction in macrophages in ES associated with intense inflammatory infiltrate&#44; as in the present case&#44; has been reported&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Such a finding is mostly seen in granulomatous processes such as rheumatoid nodules and granuloma annulare&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> A common characteristic of ES&#44; present in more than 90&#37; of cases&#44; is the loss of expression of INI-1&#44; the tumor suppressor gene expressed in normal nucleated cells&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a> A minority of cases of ES&#44; as in the reported study&#44; maintains normal INI-1 expression and may be related to a more aggressive biological behavior&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">The differential diagnosis includes nodules due to infectious disease&#44; rheumatoid nodules&#44; fibrous histiocytoma&#44; and other soft tissue sarcomas&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a> In the present case&#44; the ascending trajectory of the lesions and their rapid evolution raised the hypothesis of lymphocutaneous sporotrichosis and cutaneous mycobacteriosis&#44; which were ruled out by the cultures and histopathological analysis&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Surgery remains the mainstay of treatment in localized disease&#44; with or without radiation&#44; with disease-free surgical margins being the most important prognostic factor&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;5</span></a> Chemotherapy is the treatment of choice for advanced disease&#46; Recently&#44; tazemetostat&#44; a histone methyltransferase inhibitor&#44; was approved for the treatment of locally advanced or metastatic ES&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Financial support</span><p id="par0025" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Authors&#8217; contributions</span><p id="par0030" class="elsevierStylePara elsevierViewall">Vivian Spanemberg Macuglia&#58; Approval of the final version of the manuscript&#59; design and planning of the study&#59; drafting and editing of the manuscript&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Juliano Peruzzo&#58; Approval of the final version of the manuscript&#59; design and planning of the study&#59; drafting and editing of the manuscript&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Ariane Bastos Geller&#58; Approval of the final version of the manuscript&#59; design and planning of the study&#59; drafting and editing of the manuscript&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Renan Rangel Bonamigo&#58; Approval of the final version of the manuscript&#59; design and planning of the study&#59; drafting and editing of the manuscript&#59; intellectual participation in the propaedeutic and&#47;or therapeutic conduct of the studied cases&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0050" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span></span>"
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