<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>2341-4545</journal-id>
<journal-title><![CDATA[GE-Portuguese Journal of Gastroenterology]]></journal-title>
<abbrev-journal-title><![CDATA[GE Port J Gastroenterol]]></abbrev-journal-title>
<issn>2341-4545</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Gastrenterologia]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2341-45452024000100054</article-id>
<article-id pub-id-type="doi">10.1159/000528121</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Embryonal Sarcoma of the Liver in the Adult: Challenges in the Diagnosis of a Rare Entity]]></article-title>
<article-title xml:lang="pt"><![CDATA[Sarcoma embrionário do fígado no adulto - desafios no diagnóstico de uma entidade rara]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Fontinha]]></surname>
<given-names><![CDATA[Guilherme Nogueira]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gama]]></surname>
<given-names><![CDATA[João Martins]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Oliveira]]></surname>
<given-names><![CDATA[Rui Caetano]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cipriano]]></surname>
<given-names><![CDATA[Augusta]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Centro Hospitalar e Universitário de Coimbra Serviço de Anatomia Patológica ]]></institution>
<addr-line><![CDATA[Coimbra ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>28</day>
<month>02</month>
<year>2024</year>
</pub-date>
<pub-date pub-type="epub">
<day>28</day>
<month>02</month>
<year>2024</year>
</pub-date>
<volume>31</volume>
<numero>1</numero>
<fpage>54</fpage>
<lpage>59</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S2341-45452024000100054&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S2341-45452024000100054&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S2341-45452024000100054&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract  Introduction: Embryonal sarcoma of the liver (ESL) is a rare neoplasm of the liver occurring mainly in paediatric ages. Making the correct diagnosis can be challenging as the laboratory and radiological findings that are often nonspecific, and the tumour immunophenotype is poorly defined and even somewhat variable.  Case Presentation:  A large epigastric mass was detected in a computerized tomography scan of a 43-year-old woman presenting with abdominal pain and bloating. The mass was biopsied and submitted to histo-pathological study. Microscopically the tumour had sarcomatoid features and showed multinucleated cells with periodic acid-Schiff (PAS)-positive globules. Immunostaining revealed positivity for vimentin, CD10, glypican-3, and &#945;1-antitrypsin and negativity for keratins, muscle, adipocytic, and melanocytic differentiation markers. The patient was then submitted to a left hepatectomy with similar histological findings.  Discussion: ESL in adults is a rarity and its diagnosis requires the exclusion of other entities. While some microscopic features are very common, they remain nonspecific. The main feature is the presence of multinucleated cells with PAS-positive hyaline globules. While ancillary testing is key, the immunophenotype also lacks specificity and ESL may have variable staining for glypican-3 and epithelial or muscle differentiation markers. Although it has been described for more than 3 decades, the prognosis and optimal treatment are still not well defined, but surgery has yielded favourable results.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo  Introdução:  O sarcoma embrionário do fígado (SEF) é uma neoplasia rara do fígado que ocorre principalmente em idades pediátricas. Fazer o diagnóstico correto pode ser um desafio, uma vez que os achados laboratoriais e radiológicos são muitas vezes inespecíficos e o imunofenótipo desta entidade é mal definido e algo variável.  Apresentação do caso:  Foi detetada em tomografia computorizada (CT) abdominal uma massa epigástrica volumosa numa mulher de 43 anos apresentando dor abdom-inal e distensão abdominal. A massa foi biopsada e submetida a estudo histopatológico. Microscopicamente, o tumor tinha características sarcomatoides e apresentava células multinucleadas com glóbulos hialinos com positividade para ácido periódico Schiff (APS). O estudo imunohistoquímico revelou positividade para vimentina, CD10, glipicano-3 e &#945;1-antitripsina e negatividade para queratinas e marcadores de diferenciação muscular, adipocítica e melanocítica.  Discussão/Conclusão:  O SEF no adulto é uma raridade e o seu diagnóstico requer a ex-clusão de outras entidades. Embora algumas características microscópicas sejam muito comuns, estas permane-cem inespecíficas. A principal característica é a presença de células multinucleadas com glóbulos hialinos positivos para APS. Ainda que o estudo imunohistoquímico seja fundamental, o imunofenótipo também carece de especificidade e o SEF pode ter marcação variável para glipicano-3 e marcadores de diferenciação epitelial ou muscular. Apesar de ter sido descrito há mais de três décadas, o prognóstico e o tratamento ideal ainda não estão bem definidos, mas a cirurgia tem apresentado resultados favoráveis.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Sarcoma]]></kwd>
<kwd lng="en"><![CDATA[Embrionary]]></kwd>
<kwd lng="en"><![CDATA[Liver]]></kwd>
<kwd lng="en"><![CDATA[Adult]]></kwd>
<kwd lng="pt"><![CDATA[Sarcoma]]></kwd>
<kwd lng="pt"><![CDATA[Embrionário]]></kwd>
<kwd lng="pt"><![CDATA[Fígado]]></kwd>
<kwd lng="pt"><![CDATA[Adulto]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<label>1</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lin]]></surname>
<given-names><![CDATA[YH]]></given-names>
</name>
<name>
<surname><![CDATA[Lin]]></surname>
<given-names><![CDATA[CC]]></given-names>
</name>
<name>
<surname><![CDATA[Concejero]]></surname>
<given-names><![CDATA[AM]]></given-names>
</name>
<name>
<surname><![CDATA[Yong]]></surname>
<given-names><![CDATA[CC]]></given-names>
</name>
<name>
<surname><![CDATA[Kuo]]></surname>
<given-names><![CDATA[FY]]></given-names>
</name>
<name>
<surname><![CDATA[Wang]]></surname>
<given-names><![CDATA[CC]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Surgical experience of adult primary hepatic sarcomas]]></article-title>
<source><![CDATA[World J Surg Oncol]]></source>
<year>2015</year>
<volume>2813</volume>
<page-range>87</page-range></nlm-citation>
</ref>
<ref id="B2">
<label>2</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Martins]]></surname>
<given-names><![CDATA[ACA]]></given-names>
</name>
<name>
<surname><![CDATA[Costa Neto]]></surname>
<given-names><![CDATA[DCD]]></given-names>
</name>
<name>
<surname><![CDATA[Silva]]></surname>
<given-names><![CDATA[JDDE]]></given-names>
</name>
<name>
<surname><![CDATA[Moraes]]></surname>
<given-names><![CDATA[YM]]></given-names>
</name>
<name>
<surname><![CDATA[Leão]]></surname>
<given-names><![CDATA[CS]]></given-names>
</name>
<name>
<surname><![CDATA[Martins]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Adult primary liver sarcoma systematic review]]></article-title>
<source><![CDATA[Ver Col Bras Cir]]></source>
<year>2020</year>
<volume>47</volume>
</nlm-citation>
</ref>
<ref id="B3">
<label>3</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Putra]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Ornvold]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Undifferentiated embryonal sarcoma of the liver a concise review]]></article-title>
<source><![CDATA[Arch Pathol Lab Med]]></source>
<year>2015</year>
<volume>139</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>269-73</page-range></nlm-citation>
</ref>
<ref id="B4">
<label>4</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Manabe]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Uojima]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Hidaka]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Shao]]></surname>
<given-names><![CDATA[X]]></given-names>
</name>
<name>
<surname><![CDATA[Iwasaki]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Wada]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Undifferentiated embryonal sarcoma of the liver identified after the initial diagnosis of a hepatic cyst]]></article-title>
<source><![CDATA[Intern Med]]></source>
<year>2020</year>
<volume>59</volume>
<numero>19</numero>
<issue>19</issue>
<page-range>2375-82</page-range></nlm-citation>
</ref>
<ref id="B5">
<label>5</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Buetow]]></surname>
<given-names><![CDATA[PC]]></given-names>
</name>
<name>
<surname><![CDATA[Buck]]></surname>
<given-names><![CDATA[JL]]></given-names>
</name>
<name>
<surname><![CDATA[Pantongrag-Brown]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Marshall]]></surname>
<given-names><![CDATA[WH]]></given-names>
</name>
<name>
<surname><![CDATA[Ros]]></surname>
<given-names><![CDATA[PR]]></given-names>
</name>
<name>
<surname><![CDATA[Levine]]></surname>
<given-names><![CDATA[MS]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Undifferentiated (embryonal) sarcoma of the liver pathologic basis of imaging findings in 28 cases]]></article-title>
<source><![CDATA[Radiology]]></source>
<year>1997</year>
<volume>203</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>779-83</page-range></nlm-citation>
</ref>
<ref id="B6">
<label>6</label><nlm-citation citation-type="book">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Nagtegaal]]></surname>
<given-names><![CDATA[ID]]></given-names>
</name>
<name>
<surname><![CDATA[Odze]]></surname>
<given-names><![CDATA[RD]]></given-names>
</name>
<name>
<surname><![CDATA[Klimstra]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Paradis]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Rugge]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Schirmacher]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Mesenchymal tumours of the digestive system]]></article-title>
<person-group person-group-type="editor">
<name>
<surname><![CDATA[Hornick]]></surname>
<given-names><![CDATA[JL]]></given-names>
</name>
<name>
<surname><![CDATA[Miettinen]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Tsui]]></surname>
<given-names><![CDATA[WM]]></given-names>
</name>
</person-group>
<source><![CDATA[WHO classification of tumours]]></source>
<year>2019</year>
<edition>5</edition>
<page-range>497-8</page-range><publisher-loc><![CDATA[Lyon ]]></publisher-loc>
<publisher-name><![CDATA[IARC]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B7">
<label>7</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kuo]]></surname>
<given-names><![CDATA[LM]]></given-names>
</name>
<name>
<surname><![CDATA[Chou]]></surname>
<given-names><![CDATA[HS]]></given-names>
</name>
<name>
<surname><![CDATA[Chan]]></surname>
<given-names><![CDATA[KM]]></given-names>
</name>
<name>
<surname><![CDATA[Yu]]></surname>
<given-names><![CDATA[MC]]></given-names>
</name>
<name>
<surname><![CDATA[Lee]]></surname>
<given-names><![CDATA[WC]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A case of huge primary liposarcoma in the liver]]></article-title>
<source><![CDATA[World J Gastroenterol]]></source>
<year>2006</year>
<volume>12</volume>
<numero>7</numero>
<issue>7</issue>
<page-range>1157-9</page-range></nlm-citation>
</ref>
<ref id="B8">
<label>8</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Yasir]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Torbenson]]></surname>
<given-names><![CDATA[MS]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Angiosarcoma of the liver clinicopathologic features and morphologic patterns]]></article-title>
<source><![CDATA[Am J Surg Pathol]]></source>
<year>2019</year>
<volume>43</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>581-90</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Gong]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Li]]></surname>
<given-names><![CDATA[YH]]></given-names>
</name>
<name>
<surname><![CDATA[Zhao]]></surname>
<given-names><![CDATA[JY]]></given-names>
</name>
<name>
<surname><![CDATA[Wang]]></surname>
<given-names><![CDATA[XX]]></given-names>
</name>
<name>
<surname><![CDATA[Zhu]]></surname>
<given-names><![CDATA[SJ]]></given-names>
</name>
<name>
<surname><![CDATA[Zhang]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Primary malignant melanoma of the liver a case report]]></article-title>
<source><![CDATA[World J Gastroenterol]]></source>
<year>2008</year>
<volume>14</volume>
<numero>31</numero>
<issue>31</issue>
<page-range>4968-71</page-range></nlm-citation>
</ref>
<ref id="B10">
<label>10</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Konstantinidis]]></surname>
<given-names><![CDATA[IT]]></given-names>
</name>
<name>
<surname><![CDATA[Nota]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Jutric]]></surname>
<given-names><![CDATA[Z]]></given-names>
</name>
<name>
<surname><![CDATA[Ituarte]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Chow]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
<name>
<surname><![CDATA[Chu]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Primary liver sarcomas in the modern era resection or transplantation?]]></article-title>
<source><![CDATA[J Surg Oncol]]></source>
<year>2018</year>
<volume>117</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>886-91</page-range></nlm-citation>
</ref>
<ref id="B11">
<label>11</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Wu]]></surname>
<given-names><![CDATA[Z]]></given-names>
</name>
<name>
<surname><![CDATA[Wei]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Cai]]></surname>
<given-names><![CDATA[Z]]></given-names>
</name>
<name>
<surname><![CDATA[Zhou]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Long-term survival outcomes of undifferentiated embryonal sarcoma of the liver a pooled analysis of 308 patients]]></article-title>
<source><![CDATA[ANZ J Surg]]></source>
<year>2020</year>
<volume>90</volume>
<numero>9</numero>
<issue>9</issue>
<page-range>1615-20</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
