<?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>0123-9015</journal-id>
<journal-title><![CDATA[Revista Colombiana de Cancerología]]></journal-title>
<abbrev-journal-title><![CDATA[rev.colomb.cancerol.]]></abbrev-journal-title>
<issn>0123-9015</issn>
<publisher>
<publisher-name><![CDATA[Instituto Nacional de Cancerología E.S.E.]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0123-90152022000100111</article-id>
<article-id pub-id-type="doi">10.35509/01239015.720</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Angiosarcoma cutáneo en una adolescente con Xeroderma Pigmentoso: Reporte de caso]]></article-title>
<article-title xml:lang="en"><![CDATA[Cutaneous angiosarcoma in an adolescent girl with Xeroderma Pigmentosum: A case report]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Prada]]></surname>
<given-names><![CDATA[Camila]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Liendo]]></surname>
<given-names><![CDATA[Jonathan]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Suarez-Mattos]]></surname>
<given-names><![CDATA[Amaranto]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gnecco]]></surname>
<given-names><![CDATA[Juan P.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Melo]]></surname>
<given-names><![CDATA[Mario]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Calderón]]></surname>
<given-names><![CDATA[Alejandra]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Instituto Nacional de Cancerología Hemato-oncología Pediátrica ]]></institution>
<addr-line><![CDATA[Bogotá ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Instituto Nacional de Cancerología Cirugía de Cabeza y Cuello ]]></institution>
<addr-line><![CDATA[Bogotá ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Instituto Nacional de Cancerología Patología ]]></institution>
<addr-line><![CDATA[Bogotá ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Universidad Militar Nueva Granada  ]]></institution>
<addr-line><![CDATA[Bogotá ]]></addr-line>
<country>Colombia</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>03</month>
<year>2022</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>03</month>
<year>2022</year>
</pub-date>
<volume>26</volume>
<numero>1</numero>
<fpage>111</fpage>
<lpage>116</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_arttext&amp;pid=S0123-90152022000100111&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_abstract&amp;pid=S0123-90152022000100111&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_pdf&amp;pid=S0123-90152022000100111&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen El Xeroderma Pigmentoso (XP) es un trastorno poco frecuente, autosómico recesivo y caracterizado por una extrema sensibilidad a la radiación ultravioleta (RUV) de la luz solar, que produce una reparación defectuosa del daño del ADN y como consecuencia, marcada predisposición al desarrollo de cáncer de piel. Clínicamente, se manifiesta por fotosensibilidad, cambios cutáneos pigmentarios y envejecimiento prematuro de la piel. Este trastorno afecta aproximadamente 1 de cada 250.000 habitantes por año en Estados Unidos. Presentamos el caso de una paciente de 12 años con un Xeroderma Pigmentoso detectado a los 6 meses, quien desarrolló una lesión ulcerada en la vertiente nasal izquierda con reporte de patología de Angiosarcoma (AS) con CD31, EGR y CD34 positivos. La paciente fue tratada con resección amplia de la lesión y quimioterapia adyuvante con Plaquitaxel y Doxorrubicina sin radioterapia.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract Xeroderma Pigmentosum is a rare autosomal recessive disorder characterized by extreme sensitivity to ultraviolet radiation (UVR) from sunlight that results in a defective repair of DNA damage and, as a consequence, a marked predisposition to the development of cancer of the skin. Its clinical manifestations are photosensitivity, pigmentary skin changes, and premature aging of the skin. This disorder affects approximately 1 in 250,000 individuals per year in the United States. We present the case of a 12-year-old patient with Xeroderma Pigmentosum detected at 6 months of age, who developed an ulcerated lesion on the left nasal slope with a pathology report of angiosarcoma (AS), which we positive for ERG, CD31, and CD34. The patient was treated with wide resection of the lesion and adjuvant chemotherapy with paclitaxel and doxorubicin without radiation therapy.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[xeroderma pigmentoso]]></kwd>
<kwd lng="es"><![CDATA[angiosarcoma]]></kwd>
<kwd lng="es"><![CDATA[niños]]></kwd>
<kwd lng="es"><![CDATA[radioterapia]]></kwd>
<kwd lng="es"><![CDATA[quimioterapia]]></kwd>
<kwd lng="en"><![CDATA[Xeroderma Pigmentosum]]></kwd>
<kwd lng="en"><![CDATA[angiosarcoma]]></kwd>
<kwd lng="en"><![CDATA[children]]></kwd>
<kwd lng="en"><![CDATA[radiation therapy]]></kwd>
<kwd lng="en"><![CDATA[chemotherapy]]></kwd>
</kwd-group>
</article-meta>
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