Cutaneous Epithelioid Clear Cells Angiosarcoma in a Young Woman with Congenital Lymphedema
Identifieur interne : 003D10 ( Pmc/Curation ); précédent : 003D09; suivant : 003D11Cutaneous Epithelioid Clear Cells Angiosarcoma in a Young Woman with Congenital Lymphedema
Auteurs : Flore Tabareau-Delalande [France] ; Anne De Muret [France] ; Elodie Miquelestorena-Standley [France] ; Anne-Valérie Decouvelaere [France] ; Gonzague De Pinieux [France]Source :
- Case Reports in Pathology [ 2090-6781 ] ; 2013.
Abstract
Angiosarcomas are rare aggressive neoplasms that can occur secondary to chronic lymphedema (Stewart-Treves syndrome). Although secondary angiosarcomas are commonly described after-mastectomy and/or after-radiotherapy, few cases have been reported in association with chronic lymphedema of congenital origin. We report the clinical, pathological, and cytogenetic findings in a case of cutaneous epithelioid clear cells angiosarcoma that occurred in a 21-year-old woman with hemibody congenital lymphedema. Surgical biopsies of the tumor mass revealed diffuse epithelioid proliferation of clear atypical cells, for which immunophenotyping highlighted the vascular differentiation. Despite
Url:
DOI: 10.1155/2013/931973
PubMed: 24078891
PubMed Central: 3776547
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PMC:3776547Le document en format XML
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Cutaneous Epithelioid Clear Cells Angiosarcoma in a Young Woman with Congenital Lymphedema</title>
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<front><div type="abstract" xml:lang="en"><p>Angiosarcomas are rare aggressive neoplasms that can occur secondary to chronic lymphedema (Stewart-Treves syndrome). Although secondary angiosarcomas are commonly described after-mastectomy and/or after-radiotherapy, few cases have been reported in association with chronic lymphedema of congenital origin. We report the clinical, pathological, and cytogenetic findings in a case of cutaneous epithelioid clear cells angiosarcoma that occurred in a 21-year-old woman with hemibody congenital lymphedema. Surgical biopsies of the tumor mass revealed diffuse epithelioid proliferation of clear atypical cells, for which immunophenotyping highlighted the vascular differentiation. Despite <italic>en bloc</italic>
resection of the tumor, the patient died of metastatic disease three months after diagnosis. This case illustrates the clinical and pathology characteristics of angiosarcoma that is a rare entity secondary to chronic lymphedema. It is the first reported case for which the <italic>c-MYC</italic>
amplification status was assessed. The diagnostic value of this amplification should be further evaluated in this specific context.</p>
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<front><journal-meta><journal-id journal-id-type="nlm-ta">Case Rep Pathol</journal-id>
<journal-id journal-id-type="iso-abbrev">Case Rep Pathol</journal-id>
<journal-id journal-id-type="publisher-id">CRIM.PATHOLOGY</journal-id>
<journal-title-group><journal-title>Case Reports in Pathology</journal-title>
</journal-title-group>
<issn pub-type="ppub">2090-6781</issn>
<issn pub-type="epub">2090-679X</issn>
<publisher><publisher-name>Hindawi Publishing Corporation</publisher-name>
</publisher>
</journal-meta>
<article-meta><article-id pub-id-type="pmid">24078891</article-id>
<article-id pub-id-type="pmc">3776547</article-id>
<article-id pub-id-type="doi">10.1155/2013/931973</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Case Report</subject>
</subj-group>
</article-categories>
<title-group><article-title>Cutaneous Epithelioid Clear Cells Angiosarcoma in a Young Woman with Congenital Lymphedema</article-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Tabareau-Delalande</surname>
<given-names>Flore</given-names>
</name>
<xref ref-type="aff" rid="I1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>de Muret</surname>
<given-names>Anne</given-names>
</name>
<xref ref-type="aff" rid="I1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Miquelestorena-Standley</surname>
<given-names>Elodie</given-names>
</name>
<xref ref-type="aff" rid="I1"><sup>1</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Decouvelaere</surname>
<given-names>Anne-Valérie</given-names>
</name>
<xref ref-type="aff" rid="I2"><sup>2</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>de Pinieux</surname>
<given-names>Gonzague</given-names>
</name>
<xref ref-type="aff" rid="I1"><sup>1</sup>
</xref>
<xref ref-type="corresp" rid="cor1">*</xref>
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<aff id="I1"><sup>1</sup>
Department of Pathology, Tours University Hospital, 37044 Tours, France</aff>
<aff id="I2"><sup>2</sup>
Department of Biopathology, Léon Bérard Center, 69008 Lyon, France</aff>
<author-notes><corresp id="cor1">*Gonzague de Pinieux: <email>depinieux@med.univ-tours.fr</email>
</corresp>
<fn fn-type="other"><p>Academic Editors: I. Meattini, T. Strecker, and W. P. Wang</p>
</fn>
</author-notes>
<pub-date pub-type="ppub"><year>2013</year>
</pub-date>
<pub-date pub-type="epub"><day>3</day>
<month>9</month>
<year>2013</year>
</pub-date>
<volume>2013</volume>
<elocation-id>931973</elocation-id>
<history><date date-type="received"><day>5</day>
<month>5</month>
<year>2013</year>
</date>
<date date-type="accepted"><day>1</day>
<month>8</month>
<year>2013</year>
</date>
</history>
<permissions><copyright-statement>Copyright © 2013 Flore Tabareau-Delalande et al.</copyright-statement>
<copyright-year>2013</copyright-year>
<license xlink:href="https://creativecommons.org/licenses/by/3.0/"><license-p>This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p>
</license>
</permissions>
<abstract><p>Angiosarcomas are rare aggressive neoplasms that can occur secondary to chronic lymphedema (Stewart-Treves syndrome). Although secondary angiosarcomas are commonly described after-mastectomy and/or after-radiotherapy, few cases have been reported in association with chronic lymphedema of congenital origin. We report the clinical, pathological, and cytogenetic findings in a case of cutaneous epithelioid clear cells angiosarcoma that occurred in a 21-year-old woman with hemibody congenital lymphedema. Surgical biopsies of the tumor mass revealed diffuse epithelioid proliferation of clear atypical cells, for which immunophenotyping highlighted the vascular differentiation. Despite <italic>en bloc</italic>
resection of the tumor, the patient died of metastatic disease three months after diagnosis. This case illustrates the clinical and pathology characteristics of angiosarcoma that is a rare entity secondary to chronic lymphedema. It is the first reported case for which the <italic>c-MYC</italic>
amplification status was assessed. The diagnostic value of this amplification should be further evaluated in this specific context.</p>
</abstract>
</article-meta>
</front>
<floats-group><fig id="fig1" orientation="portrait" position="float"><label>Figure 1</label>
<graphic xlink:href="CRIM.PATHOLOGY2013-931973.001"></graphic>
</fig>
<fig id="fig2" orientation="portrait" position="float"><label>Figure 2</label>
<graphic xlink:href="CRIM.PATHOLOGY2013-931973.002"></graphic>
</fig>
<table-wrap id="tab1" orientation="portrait" position="float"><label>Table 1</label>
<caption><p>Clinical features of patients with cutaneous angiosarcoma associated with congenital lymphedema described in the literature (modified from Offori et al., 1993 [<xref rid="B12" ref-type="bibr">12</xref>
] and Bernardi et al., 2009 [<xref rid="B18" ref-type="bibr">18</xref>
] ).</p>
</caption>
<table frame="hsides" rules="groups"><thead><tr><th align="left" rowspan="1" colspan="1">Author</th>
<th align="center" rowspan="1" colspan="1">Sex</th>
<th align="center" rowspan="1" colspan="1">Age at diagnosis</th>
<th align="center" rowspan="1" colspan="1">Tumor site</th>
<th align="left" rowspan="1" colspan="1">Clinical appearance</th>
<th align="center" rowspan="1" colspan="1">Interval in diagnosis</th>
<th align="left" rowspan="1" colspan="1">Treatment</th>
<th align="center" rowspan="1" colspan="1">Survival</th>
</tr>
</thead>
<tbody><tr><td align="left" rowspan="1" colspan="1">Kettle [<xref rid="B19" ref-type="bibr">19</xref>
]</td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">44 y</td>
<td align="center" rowspan="1" colspan="1">RL</td>
<td align="left" rowspan="1" colspan="1">Blue-red dislocation</td>
<td align="center" rowspan="1" colspan="1">Not stated</td>
<td align="left" rowspan="1" colspan="1">Amputation</td>
<td align="center" rowspan="1" colspan="1">Not stated</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Liszauer et al. [<xref rid="B20" ref-type="bibr">20</xref>
] </td>
<td align="center" rowspan="1" colspan="1">M</td>
<td align="center" rowspan="1" colspan="1">28 y</td>
<td align="center" rowspan="1" colspan="1">RLL</td>
<td align="left" rowspan="1" colspan="1">Ulcerated lesion</td>
<td align="center" rowspan="1" colspan="1">4 m</td>
<td align="left" rowspan="1" colspan="1">Local excision</td>
<td align="center" rowspan="1" colspan="1">Died at 11 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Scott et al. [<xref rid="B21" ref-type="bibr">21</xref>
]</td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">50 y</td>
<td align="center" rowspan="1" colspan="1">LUL</td>
<td align="left" rowspan="1" colspan="1">Papillomatous nodules</td>
<td align="center" rowspan="1" colspan="1">>7 m</td>
<td align="left" rowspan="1" colspan="1">Local excision/amputation</td>
<td align="center" rowspan="1" colspan="1">Died at 37 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Taswell et al. [<xref rid="B22" ref-type="bibr">22</xref>
] </td>
<td align="center" rowspan="1" colspan="1">M</td>
<td align="center" rowspan="1" colspan="1">17 y</td>
<td align="center" rowspan="1" colspan="1">LUL</td>
<td align="left" rowspan="1" colspan="1">Ulcerated lesion</td>
<td align="center" rowspan="1" colspan="1">Not stated</td>
<td align="left" rowspan="1" colspan="1">Disarticulation</td>
<td align="center" rowspan="1" colspan="1">Died at 24 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Bunch [<xref rid="B23" ref-type="bibr">23</xref>
] </td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">13 y</td>
<td align="center" rowspan="1" colspan="1">UL</td>
<td align="left" rowspan="1" colspan="1">Not stated</td>
<td align="center" rowspan="1" colspan="1">Not stated</td>
<td align="left" rowspan="1" colspan="1">Interscapular amputation</td>
<td align="center" rowspan="1" colspan="1">Died at 1 y</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Finlay-Jones [<xref rid="B24" ref-type="bibr">24</xref>
] </td>
<td align="center" rowspan="1" colspan="1">M</td>
<td align="center" rowspan="1" colspan="1">34 y</td>
<td align="center" rowspan="1" colspan="1">LLL</td>
<td align="left" rowspan="1" colspan="1">Ulcerated blue tumor</td>
<td align="center" rowspan="1" colspan="1">Not stated</td>
<td align="left" rowspan="1" colspan="1">Excision and combined therapy</td>
<td align="center" rowspan="1" colspan="1">Died at 31 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Merrick et al. [<xref rid="B25" ref-type="bibr">25</xref>
] </td>
<td align="center" rowspan="1" colspan="1">M</td>
<td align="center" rowspan="1" colspan="1">52 y</td>
<td align="center" rowspan="1" colspan="1">LUL</td>
<td align="left" rowspan="1" colspan="1">Swelling/blue nodules</td>
<td align="center" rowspan="1" colspan="1">6 m</td>
<td align="left" rowspan="1" colspan="1">Wide local excision/amputation/radiotherapy</td>
<td align="center" rowspan="1" colspan="1">Died at 36 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Mackenzie [<xref rid="B26" ref-type="bibr">26</xref>
] </td>
<td align="center" rowspan="1" colspan="1">M</td>
<td align="center" rowspan="1" colspan="1">64 y</td>
<td align="center" rowspan="1" colspan="1">RLL</td>
<td align="left" rowspan="1" colspan="1">Nodules/ulceration</td>
<td align="center" rowspan="1" colspan="1">2 m</td>
<td align="left" rowspan="1" colspan="1">Hindquarter amputation/radiotherapy</td>
<td align="center" rowspan="1" colspan="1">Alive at 24 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Dubin et al. [<xref rid="B27" ref-type="bibr">27</xref>
] </td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">29 y</td>
<td align="center" rowspan="1" colspan="1">LUL</td>
<td align="left" rowspan="1" colspan="1">Ulcerated lesion</td>
<td align="center" rowspan="1" colspan="1">3 y</td>
<td align="left" rowspan="1" colspan="1">Disarticulation</td>
<td align="center" rowspan="1" colspan="1">Died at 45 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Laskas et al. [<xref rid="B28" ref-type="bibr">28</xref>
]</td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">85 y</td>
<td align="center" rowspan="1" colspan="1">RUL</td>
<td align="left" rowspan="1" colspan="1">Purple papules and blisters/ulceration</td>
<td align="center" rowspan="1" colspan="1">10 m</td>
<td align="left" rowspan="1" colspan="1">Palliative midhumeral amputation</td>
<td align="center" rowspan="1" colspan="1">Died at 14 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Banathy et al. [<xref rid="B29" ref-type="bibr">29</xref>
] </td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">50 y</td>
<td align="center" rowspan="1" colspan="1">LUL</td>
<td align="left" rowspan="1" colspan="1">Purple nodules</td>
<td align="center" rowspan="1" colspan="1">Not stated</td>
<td align="left" rowspan="1" colspan="1">Midhumeral amputation</td>
<td align="center" rowspan="1" colspan="1">Died at 26 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Sordillo et al. [<xref rid="B30" ref-type="bibr">30</xref>
] </td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">23 y</td>
<td align="center" rowspan="1" colspan="1"> </td>
<td align="left" rowspan="1" colspan="1">Not stated</td>
<td align="center" rowspan="1" colspan="1">Not stated</td>
<td align="left" rowspan="1" colspan="1">Hemipelvectomy</td>
<td align="center" rowspan="1" colspan="1">Alive at 19 y</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Bostrom et al. [<xref rid="B31" ref-type="bibr">31</xref>
] </td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">19 y</td>
<td align="center" rowspan="1" colspan="1">RUL</td>
<td align="left" rowspan="1" colspan="1">Infected lesion</td>
<td align="center" rowspan="1" colspan="1">4 m</td>
<td align="left" rowspan="1" colspan="1">Amputation/disarticulation</td>
<td align="center" rowspan="1" colspan="1">Died at 12 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1"> </td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">10 y</td>
<td align="center" rowspan="1" colspan="1">LLL</td>
<td align="left" rowspan="1" colspan="1">Blue/red nodules</td>
<td align="center" rowspan="1" colspan="1">8 m</td>
<td align="left" rowspan="1" colspan="1">Amputation/disarticulation</td>
<td align="center" rowspan="1" colspan="1">Alive at 10 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Offori et al., 1993 [<xref rid="B12" ref-type="bibr">12</xref>
]</td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">43 y</td>
<td align="center" rowspan="1" colspan="1">LLL</td>
<td align="left" rowspan="1" colspan="1">Blue/purple nodules</td>
<td align="center" rowspan="1" colspan="1">2 m</td>
<td align="left" rowspan="1" colspan="1">Amputation</td>
<td align="center" rowspan="1" colspan="1">Alive at 9 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Andersson et al. [<xref rid="B32" ref-type="bibr">32</xref>
] </td>
<td align="center" rowspan="1" colspan="1">M</td>
<td align="center" rowspan="1" colspan="1">35 y</td>
<td align="center" rowspan="1" colspan="1">RL</td>
<td align="left" rowspan="1" colspan="1">Blue nodules</td>
<td align="center" rowspan="1" colspan="1">Not stated</td>
<td align="left" rowspan="1" colspan="1">Hemipelvectomy, chemotherapy</td>
<td align="center" rowspan="1" colspan="1">Not stated</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Cerri et al. [<xref rid="B33" ref-type="bibr">33</xref>
] </td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">42 y</td>
<td align="center" rowspan="1" colspan="1">Pubis</td>
<td align="left" rowspan="1" colspan="1">Ecchymotic plaque</td>
<td align="center" rowspan="1" colspan="1">Not stated</td>
<td align="left" rowspan="1" colspan="1">Hemipelvectomy, radiotherapy</td>
<td align="center" rowspan="1" colspan="1">Not stated</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Bernardi et al. [<xref rid="B18" ref-type="bibr">18</xref>
]</td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">4 y</td>
<td align="center" rowspan="1" colspan="1">LLL</td>
<td align="left" rowspan="1" colspan="1">Ulcerated lesion/blue nodules</td>
<td align="center" rowspan="1" colspan="1">1 y</td>
<td align="left" rowspan="1" colspan="1">Local excision</td>
<td align="center" rowspan="1" colspan="1">Alive at 14 m</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Deyrup et al., 2011 [<xref rid="B14" ref-type="bibr">14</xref>
]</td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">2 y</td>
<td align="center" rowspan="1" colspan="1">Foot</td>
<td align="left" rowspan="1" colspan="1">Not stated</td>
<td align="center" rowspan="1" colspan="1">Not stated</td>
<td align="left" rowspan="1" colspan="1">Local excision</td>
<td align="center" rowspan="1" colspan="1">Died at 1 y</td>
</tr>
<tr><td align="left" rowspan="1" colspan="1">Present case</td>
<td align="center" rowspan="1" colspan="1">F</td>
<td align="center" rowspan="1" colspan="1">21 y</td>
<td align="center" rowspan="1" colspan="1">Groin</td>
<td align="left" rowspan="1" colspan="1">Purple/reddish infiltrated and ulcerated mass</td>
<td align="center" rowspan="1" colspan="1">10 m</td>
<td align="left" rowspan="1" colspan="1">Local excision</td>
<td align="center" rowspan="1" colspan="1">Died at 3 m</td>
</tr>
</tbody>
</table>
<table-wrap-foot><fn><p>LLL: left lower limb; RLL: right lower limb; LUL: left upper limb; RUL: right upper limb; m: months; and y: years.</p>
</fn>
</table-wrap-foot>
</table-wrap>
</floats-group>
</pmc>
</record>
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