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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Angiosarcoma Arising from the Tongue of an 11-Year-Old Girl with Xeroderma Pigmentosum</title>
<author><name sortKey="Olson, Matthew T" sort="Olson, Matthew T" uniqKey="Olson M" first="Matthew T." last="Olson">Matthew T. Olson</name>
<affiliation><nlm:aff id="Aff1">Departments of Pathology, The Johns Hopkins University School of Medicine, 401 N. Broadway, Weinberg 2242, Baltimore, MD 21231 USA</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Puttgen, Katherine B" sort="Puttgen, Katherine B" uniqKey="Puttgen K" first="Katherine B." last="Puttgen">Katherine B. Puttgen</name>
<affiliation><nlm:aff id="Aff2">Departments of Dermatology, The Johns Hopkins Medical Institutions, Baltimore, MD USA</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Westra, William H" sort="Westra, William H" uniqKey="Westra W" first="William H." last="Westra">William H. Westra</name>
<affiliation><nlm:aff id="Aff1">Departments of Pathology, The Johns Hopkins University School of Medicine, 401 N. Broadway, Weinberg 2242, Baltimore, MD 21231 USA</nlm:aff>
</affiliation>
<affiliation><nlm:aff id="Aff2">Departments of Dermatology, The Johns Hopkins Medical Institutions, Baltimore, MD USA</nlm:aff>
</affiliation>
<affiliation><nlm:aff id="Aff3">Departments of Oncology, The Johns Hopkins Medical Institutions, Baltimore, MD USA</nlm:aff>
</affiliation>
<affiliation><nlm:aff id="Aff4">Departments of Otolaryngology/Head and Neck Surgery, The Johns Hopkins Medical Institutions, Baltimore, MD USA</nlm:aff>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">PMC</idno>
<idno type="pmid">21987224</idno>
<idno type="pmc">3370034</idno>
<idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3370034</idno>
<idno type="RBID">PMC:3370034</idno>
<idno type="doi">10.1007/s12105-011-0303-x</idno>
<date when="2011">2011</date>
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Angiosarcoma Arising from the Tongue of an 11-Year-Old Girl with Xeroderma Pigmentosum</title>
<author><name sortKey="Olson, Matthew T" sort="Olson, Matthew T" uniqKey="Olson M" first="Matthew T." last="Olson">Matthew T. Olson</name>
<affiliation><nlm:aff id="Aff1">Departments of Pathology, The Johns Hopkins University School of Medicine, 401 N. Broadway, Weinberg 2242, Baltimore, MD 21231 USA</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Puttgen, Katherine B" sort="Puttgen, Katherine B" uniqKey="Puttgen K" first="Katherine B." last="Puttgen">Katherine B. Puttgen</name>
<affiliation><nlm:aff id="Aff2">Departments of Dermatology, The Johns Hopkins Medical Institutions, Baltimore, MD USA</nlm:aff>
</affiliation>
</author>
<author><name sortKey="Westra, William H" sort="Westra, William H" uniqKey="Westra W" first="William H." last="Westra">William H. Westra</name>
<affiliation><nlm:aff id="Aff1">Departments of Pathology, The Johns Hopkins University School of Medicine, 401 N. Broadway, Weinberg 2242, Baltimore, MD 21231 USA</nlm:aff>
</affiliation>
<affiliation><nlm:aff id="Aff2">Departments of Dermatology, The Johns Hopkins Medical Institutions, Baltimore, MD USA</nlm:aff>
</affiliation>
<affiliation><nlm:aff id="Aff3">Departments of Oncology, The Johns Hopkins Medical Institutions, Baltimore, MD USA</nlm:aff>
</affiliation>
<affiliation><nlm:aff id="Aff4">Departments of Otolaryngology/Head and Neck Surgery, The Johns Hopkins Medical Institutions, Baltimore, MD USA</nlm:aff>
</affiliation>
</author>
</analytic>
<series><title level="j">Head and Neck Pathology</title>
<idno type="ISSN">1936-055X</idno>
<idno type="eISSN">1936-0568</idno>
<imprint><date when="2011">2011</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass></textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en"><p>Xeroderma pigmentosum (XP) is a rare autosomal recessive defect in DNA endonuclease activity that is associated with the development of cutaneous malignancies, at sun exposed sites, including basal cell carcinoma, squamous cell carcinoma, and melanoma. Squamous cell carcinomas are also known to target the anterior tongue. Patients sometimes develop angiosarcomas, and these invariably arise from sun-exposed skin. A biopsy was taken from a large mass arising in the anterior tongue of an 11-year-old girl with XP and a history of cutaneous basal cell carcinomas. The histopathologic findings demonstrated a high grade epithelioid neoplasm resembling a poorly differentiated squamous cell carcinoma, but the immunohistochemical profile (AE1/AE3 negative, p63 negative, CD31 positive, CD34 positive) established the diagnosis of angiosarcoma. Angiosarcoma is an XP-related tumor that usually arises in sun-exposed skin but can also arise in the oral cavity. For patients with XP who develop epithelioid neoplasms of the oral cavity, epithelioid angiosarcoma should be considered in the differential diagnosis.</p>
</div>
</front>
</TEI>
<pmc article-type="case-report"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Head Neck Pathol</journal-id>
<journal-id journal-id-type="iso-abbrev">Head Neck Pathol</journal-id>
<journal-title-group><journal-title>Head and Neck Pathology</journal-title>
</journal-title-group>
<issn pub-type="ppub">1936-055X</issn>
<issn pub-type="epub">1936-0568</issn>
<publisher><publisher-name>Springer US</publisher-name>
<publisher-loc>Boston</publisher-loc>
</publisher>
</journal-meta>
<article-meta><article-id pub-id-type="pmid">21987224</article-id>
<article-id pub-id-type="pmc">3370034</article-id>
<article-id pub-id-type="publisher-id">303</article-id>
<article-id pub-id-type="doi">10.1007/s12105-011-0303-x</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Case Report</subject>
</subj-group>
</article-categories>
<title-group><article-title>Angiosarcoma Arising from the Tongue of an 11-Year-Old Girl with Xeroderma Pigmentosum</article-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Olson</surname>
<given-names>Matthew T.</given-names>
</name>
<xref ref-type="aff" rid="Aff1">1</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Puttgen</surname>
<given-names>Katherine B.</given-names>
</name>
<xref ref-type="aff" rid="Aff2">2</xref>
</contrib>
<contrib contrib-type="author" corresp="yes"><name><surname>Westra</surname>
<given-names>William H.</given-names>
</name>
<address><phone>+1-410-9552163</phone>
<fax>+1-410-9550115</fax>
<email>wwestra@jhmi.edu</email>
</address>
<xref ref-type="aff" rid="Aff1">1</xref>
<xref ref-type="aff" rid="Aff2">2</xref>
<xref ref-type="aff" rid="Aff3">3</xref>
<xref ref-type="aff" rid="Aff4">4</xref>
</contrib>
<aff id="Aff1"><label>1</label>
Departments of Pathology, The Johns Hopkins University School of Medicine, 401 N. Broadway, Weinberg 2242, Baltimore, MD 21231 USA</aff>
<aff id="Aff2"><label>2</label>
Departments of Dermatology, The Johns Hopkins Medical Institutions, Baltimore, MD USA</aff>
<aff id="Aff3"><label>3</label>
Departments of Oncology, The Johns Hopkins Medical Institutions, Baltimore, MD USA</aff>
<aff id="Aff4"><label>4</label>
Departments of Otolaryngology/Head and Neck Surgery, The Johns Hopkins Medical Institutions, Baltimore, MD USA</aff>
</contrib-group>
<pub-date pub-type="epub"><day>11</day>
<month>10</month>
<year>2011</year>
</pub-date>
<pub-date pub-type="collection"><month>6</month>
<year>2012</year>
</pub-date>
<volume>6</volume>
<issue>2</issue>
<fpage>255</fpage>
<lpage>257</lpage>
<history><date date-type="received"><day>18</day>
<month>7</month>
<year>2011</year>
</date>
<date date-type="accepted"><day>26</day>
<month>9</month>
<year>2011</year>
</date>
</history>
<permissions><copyright-statement>© Springer Science+Business Media, LLC 2011</copyright-statement>
</permissions>
<abstract id="Abs1"><p>Xeroderma pigmentosum (XP) is a rare autosomal recessive defect in DNA endonuclease activity that is associated with the development of cutaneous malignancies, at sun exposed sites, including basal cell carcinoma, squamous cell carcinoma, and melanoma. Squamous cell carcinomas are also known to target the anterior tongue. Patients sometimes develop angiosarcomas, and these invariably arise from sun-exposed skin. A biopsy was taken from a large mass arising in the anterior tongue of an 11-year-old girl with XP and a history of cutaneous basal cell carcinomas. The histopathologic findings demonstrated a high grade epithelioid neoplasm resembling a poorly differentiated squamous cell carcinoma, but the immunohistochemical profile (AE1/AE3 negative, p63 negative, CD31 positive, CD34 positive) established the diagnosis of angiosarcoma. Angiosarcoma is an XP-related tumor that usually arises in sun-exposed skin but can also arise in the oral cavity. For patients with XP who develop epithelioid neoplasms of the oral cavity, epithelioid angiosarcoma should be considered in the differential diagnosis.</p>
</abstract>
<kwd-group><title>Keywords</title>
<kwd>Xeroderma pigmentosum</kwd>
<kwd>Angiosarcoma</kwd>
<kwd>Head and neck cancer</kwd>
<kwd>Squamous cell carcinoma</kwd>
</kwd-group>
<custom-meta-group><custom-meta><meta-name>issue-copyright-statement</meta-name>
<meta-value>© Springer Science+Business Media, LLC 2012</meta-value>
</custom-meta>
</custom-meta-group>
</article-meta>
</front>
</pmc>
</record>
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