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
Links toward previous steps (curation, corpus...)
- to stream Pmc, to step Corpus: Pour aller vers cette notice dans l'étape Curation :003D11
Links to Exploration step
PMC:3776547Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Cutaneous Epithelioid Clear Cells Angiosarcoma in a Young Woman with Congenital Lymphedema</title>
<author><name sortKey="Tabareau Delalande, Flore" sort="Tabareau Delalande, Flore" uniqKey="Tabareau Delalande F" first="Flore" last="Tabareau-Delalande">Flore Tabareau-Delalande</name>
<affiliation wicri:level="1"><nlm:aff id="I1">Department of Pathology, Tours University Hospital, 37044 Tours, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Department of Pathology, Tours University Hospital, 37044 Tours</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="De Muret, Anne" sort="De Muret, Anne" uniqKey="De Muret A" first="Anne" last="De Muret">Anne De Muret</name>
<affiliation wicri:level="1"><nlm:aff id="I1">Department of Pathology, Tours University Hospital, 37044 Tours, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Department of Pathology, Tours University Hospital, 37044 Tours</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Miquelestorena Standley, Elodie" sort="Miquelestorena Standley, Elodie" uniqKey="Miquelestorena Standley E" first="Elodie" last="Miquelestorena-Standley">Elodie Miquelestorena-Standley</name>
<affiliation wicri:level="1"><nlm:aff id="I1">Department of Pathology, Tours University Hospital, 37044 Tours, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Department of Pathology, Tours University Hospital, 37044 Tours</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Decouvelaere, Anne Valerie" sort="Decouvelaere, Anne Valerie" uniqKey="Decouvelaere A" first="Anne-Valérie" last="Decouvelaere">Anne-Valérie Decouvelaere</name>
<affiliation wicri:level="1"><nlm:aff id="I2">Department of Biopathology, Léon Bérard Center, 69008 Lyon, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Department of Biopathology, Léon Bérard Center, 69008 Lyon</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="De Pinieux, Gonzague" sort="De Pinieux, Gonzague" uniqKey="De Pinieux G" first="Gonzague" last="De Pinieux">Gonzague De Pinieux</name>
<affiliation wicri:level="1"><nlm:aff id="I1">Department of Pathology, Tours University Hospital, 37044 Tours, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Department of Pathology, Tours University Hospital, 37044 Tours</wicri:regionArea>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">PMC</idno>
<idno type="pmid">24078891</idno>
<idno type="pmc">3776547</idno>
<idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3776547</idno>
<idno type="RBID">PMC:3776547</idno>
<idno type="doi">10.1155/2013/931973</idno>
<date when="2013">2013</date>
<idno type="wicri:Area/Pmc/Corpus">003D11</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">003D11</idno>
<idno type="wicri:Area/Pmc/Curation">003D10</idno>
<idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">003D10</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Cutaneous Epithelioid Clear Cells Angiosarcoma in a Young Woman with Congenital Lymphedema</title>
<author><name sortKey="Tabareau Delalande, Flore" sort="Tabareau Delalande, Flore" uniqKey="Tabareau Delalande F" first="Flore" last="Tabareau-Delalande">Flore Tabareau-Delalande</name>
<affiliation wicri:level="1"><nlm:aff id="I1">Department of Pathology, Tours University Hospital, 37044 Tours, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Department of Pathology, Tours University Hospital, 37044 Tours</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="De Muret, Anne" sort="De Muret, Anne" uniqKey="De Muret A" first="Anne" last="De Muret">Anne De Muret</name>
<affiliation wicri:level="1"><nlm:aff id="I1">Department of Pathology, Tours University Hospital, 37044 Tours, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Department of Pathology, Tours University Hospital, 37044 Tours</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Miquelestorena Standley, Elodie" sort="Miquelestorena Standley, Elodie" uniqKey="Miquelestorena Standley E" first="Elodie" last="Miquelestorena-Standley">Elodie Miquelestorena-Standley</name>
<affiliation wicri:level="1"><nlm:aff id="I1">Department of Pathology, Tours University Hospital, 37044 Tours, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Department of Pathology, Tours University Hospital, 37044 Tours</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Decouvelaere, Anne Valerie" sort="Decouvelaere, Anne Valerie" uniqKey="Decouvelaere A" first="Anne-Valérie" last="Decouvelaere">Anne-Valérie Decouvelaere</name>
<affiliation wicri:level="1"><nlm:aff id="I2">Department of Biopathology, Léon Bérard Center, 69008 Lyon, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Department of Biopathology, Léon Bérard Center, 69008 Lyon</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="De Pinieux, Gonzague" sort="De Pinieux, Gonzague" uniqKey="De Pinieux G" first="Gonzague" last="De Pinieux">Gonzague De Pinieux</name>
<affiliation wicri:level="1"><nlm:aff id="I1">Department of Pathology, Tours University Hospital, 37044 Tours, France</nlm:aff>
<country xml:lang="fr">France</country>
<wicri:regionArea>Department of Pathology, Tours University Hospital, 37044 Tours</wicri:regionArea>
</affiliation>
</author>
</analytic>
<series><title level="j">Case Reports in Pathology</title>
<idno type="ISSN">2090-6781</idno>
<idno type="eISSN">2090-679X</idno>
<imprint><date when="2013">2013</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass></textClass>
</profileDesc>
</teiHeader>
<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>
</div>
</front>
<back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Rouhani, P" uniqKey="Rouhani P">P Rouhani</name>
</author>
<author><name sortKey="Fletcher, Cdm" uniqKey="Fletcher C">CDM Fletcher</name>
</author>
<author><name sortKey="Devesa, Ss" uniqKey="Devesa S">SS Devesa</name>
</author>
<author><name sortKey="Toro, Jr" uniqKey="Toro J">JR Toro</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Fletcher, Jdm" uniqKey="Fletcher J">JDM Fletcher</name>
</author>
<author><name sortKey="Unni, Kk" uniqKey="Unni K">KK Unni</name>
</author>
<author><name sortKey="Mertens, E" uniqKey="Mertens E">E Mertens</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Fayette, J" uniqKey="Fayette J">J Fayette</name>
</author>
<author><name sortKey="Martin, E" uniqKey="Martin E">E Martin</name>
</author>
<author><name sortKey="Piperno Neumann, S" uniqKey="Piperno Neumann S">S Piperno-Neumann</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Strobbe, Lja" uniqKey="Strobbe L">LJA Strobbe</name>
</author>
<author><name sortKey="Peterse, Hl" uniqKey="Peterse H">HL Peterse</name>
</author>
<author><name sortKey="Van Tinteren, H" uniqKey="Van Tinteren H">H Van Tinteren</name>
</author>
<author><name sortKey="Wijnmaalen, A" uniqKey="Wijnmaalen A">A Wijnmaalen</name>
</author>
<author><name sortKey="Rutgers, Ejt" uniqKey="Rutgers E">EJT Rutgers</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Stewart, Fw" uniqKey="Stewart F">FW Stewart</name>
</author>
<author><name sortKey="Treves, N" uniqKey="Treves N">N Treves</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Styring, E" uniqKey="Styring E">E Styring</name>
</author>
<author><name sortKey="Fernebro, J" uniqKey="Fernebro J">J Fernebro</name>
</author>
<author><name sortKey="Jonsson, P" uniqKey="Jonsson P">P Jönsson</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Manner, J" uniqKey="Manner J">J Manner</name>
</author>
<author><name sortKey="Radlwimmer, B" uniqKey="Radlwimmer B">B Radlwimmer</name>
</author>
<author><name sortKey="Hohenberger, P" uniqKey="Hohenberger P">P Hohenberger</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Meis Kindblom, Jm" uniqKey="Meis Kindblom J">JM Meis-Kindblom</name>
</author>
<author><name sortKey="Kindblom, L" uniqKey="Kindblom L">L Kindblom</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Bacchi, Ce" uniqKey="Bacchi C">CE Bacchi</name>
</author>
<author><name sortKey="Silva, Tr" uniqKey="Silva T">TR Silva</name>
</author>
<author><name sortKey="Zambrano, E" uniqKey="Zambrano E">E Zambrano</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Ahrens, Wa" uniqKey="Ahrens W">WA Ahrens</name>
</author>
<author><name sortKey="Ridenour, Rv" uniqKey="Ridenour R">RV Ridenour</name>
</author>
<author><name sortKey="Caron, Bl" uniqKey="Caron B">BL Caron</name>
</author>
<author><name sortKey="Miller, Dv" uniqKey="Miller D">DV Miller</name>
</author>
<author><name sortKey="Folpe, Al" uniqKey="Folpe A">AL Folpe</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Kosemehmeto Lu, K" uniqKey="Kosemehmeto Lu K">K Kösemehmetoğlu</name>
</author>
<author><name sortKey="Gediko Lu, G" uniqKey="Gediko Lu G">G Gedikoğlu</name>
</author>
<author><name sortKey="Ruacan, " uniqKey="Ruacan ">Ş Ruacan</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Offori, Tw" uniqKey="Offori T">TW Offori</name>
</author>
<author><name sortKey="Platt, Cc" uniqKey="Platt C">CC Platt</name>
</author>
<author><name sortKey="Stephens, M" uniqKey="Stephens M">M Stephens</name>
</author>
<author><name sortKey="Hopkinson, Gb" uniqKey="Hopkinson G">GB Hopkinson</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Deyrup, At" uniqKey="Deyrup A">AT Deyrup</name>
</author>
<author><name sortKey="Mckenney, Jk" uniqKey="Mckenney J">JK McKenney</name>
</author>
<author><name sortKey="Tighiouart, M" uniqKey="Tighiouart M">M Tighiouart</name>
</author>
<author><name sortKey="Folpe, Al" uniqKey="Folpe A">AL Folpe</name>
</author>
<author><name sortKey="Weiss, Sw" uniqKey="Weiss S">SW Weiss</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Deyrup, At" uniqKey="Deyrup A">AT Deyrup</name>
</author>
<author><name sortKey="Miettinen, M" uniqKey="Miettinen M">M Miettinen</name>
</author>
<author><name sortKey="North, Pe" uniqKey="North P">PE North</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Itakura, E" uniqKey="Itakura E">E Itakura</name>
</author>
<author><name sortKey="Yamamoto, H" uniqKey="Yamamoto H">H Yamamoto</name>
</author>
<author><name sortKey="Oda, Y" uniqKey="Oda Y">Y Oda</name>
</author>
<author><name sortKey="Tsuneyoshi, M" uniqKey="Tsuneyoshi M">M Tsuneyoshi</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Mentzel, T" uniqKey="Mentzel T">T Mentzel</name>
</author>
<author><name sortKey="Schildhaus, Hu" uniqKey="Schildhaus H">HU Schildhaus</name>
</author>
<author><name sortKey="Palmedo, G" uniqKey="Palmedo G">G Palmedo</name>
</author>
<author><name sortKey="Buttner, R" uniqKey="Buttner R">R Büttner</name>
</author>
<author><name sortKey="Kutzner, H" uniqKey="Kutzner H">H Kutzner</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Fernandez, Ap" uniqKey="Fernandez A">AP Fernandez</name>
</author>
<author><name sortKey="Sun, Y" uniqKey="Sun Y">Y Sun</name>
</author>
<author><name sortKey="Tubbs, Rr" uniqKey="Tubbs R">RR Tubbs</name>
</author>
<author><name sortKey="Goldblum, Jr" uniqKey="Goldblum J">JR Goldblum</name>
</author>
<author><name sortKey="Billings, Sd" uniqKey="Billings S">SD Billings</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Bernardi, P" uniqKey="Bernardi P">P Bernardi</name>
</author>
<author><name sortKey="Zen, P" uniqKey="Zen P">P Zen</name>
</author>
<author><name sortKey="Furian, R" uniqKey="Furian R">R Furian</name>
</author>
<author><name sortKey="Medina, C" uniqKey="Medina C">C Medina</name>
</author>
<author><name sortKey="Graziadio, C" uniqKey="Graziadio C">C Graziadio</name>
</author>
<author><name sortKey="Paskulin, G" uniqKey="Paskulin G">G Paskulin</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Kettle, Eh" uniqKey="Kettle E">EH Kettle</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Liszauer, S" uniqKey="Liszauer S">S Liszauer</name>
</author>
<author><name sortKey="Ross, Rc" uniqKey="Ross R">RC Ross</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Scott, Rb" uniqKey="Scott R">RB Scott</name>
</author>
<author><name sortKey="Nydick, I" uniqKey="Nydick I">I Nydick</name>
</author>
<author><name sortKey="Conway, H" uniqKey="Conway H">H Conway</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Taswell, Hf" uniqKey="Taswell H">HF Taswell</name>
</author>
<author><name sortKey="Soule, Eh" uniqKey="Soule E">EH Soule</name>
</author>
<author><name sortKey="Coventry, Mb" uniqKey="Coventry M">MB Coventry</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Barnett, Wo" uniqKey="Barnett W">WO Barnett</name>
</author>
<author><name sortKey="Hardy, Jd" uniqKey="Hardy J">JD Hardy</name>
</author>
<author><name sortKey="Hendrix, Jh" uniqKey="Hendrix J">JH Hendrix</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Finlay Jones, Lr" uniqKey="Finlay Jones L">LR Finlay-Jones</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Merrick, Ta" uniqKey="Merrick T">TA Merrick</name>
</author>
<author><name sortKey="Erlandson, Ra" uniqKey="Erlandson R">RA Erlandson</name>
</author>
<author><name sortKey="Hajdu, Si" uniqKey="Hajdu S">SI Hajdu</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Mackenzie, Dh" uniqKey="Mackenzie D">DH Mackenzie</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Dubin, Hv" uniqKey="Dubin H">HV Dubin</name>
</author>
<author><name sortKey="Creehan, Ep" uniqKey="Creehan E">EP Creehan</name>
</author>
<author><name sortKey="Headington, Jt" uniqKey="Headington J">JT Headington</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Laskas, Jj" uniqKey="Laskas J">JJ Laskas</name>
</author>
<author><name sortKey="Shelley, Wb" uniqKey="Shelley W">WB Shelley</name>
</author>
<author><name sortKey="Wood, Mg" uniqKey="Wood M">MG Wood</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Banathy, Lj" uniqKey="Banathy L">LJ Banathy</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Sordillo, Pp" uniqKey="Sordillo P">PP Sordillo</name>
</author>
<author><name sortKey="Chapman, R" uniqKey="Chapman R">R Chapman</name>
</author>
<author><name sortKey="Hajdu, Si" uniqKey="Hajdu S">SI Hajdu</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Brostrom, L A" uniqKey="Brostrom L">L-A Brostrom</name>
</author>
<author><name sortKey="Nilsonne, U" uniqKey="Nilsonne U">U Nilsonne</name>
</author>
<author><name sortKey="Kronberg, M" uniqKey="Kronberg M">M Kronberg</name>
</author>
<author><name sortKey="Soderberg, G" uniqKey="Soderberg G">G Soderberg</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Andersson, Hc" uniqKey="Andersson H">HC Andersson</name>
</author>
<author><name sortKey="Parry, Dm" uniqKey="Parry D">DM Parry</name>
</author>
<author><name sortKey="Mulvihill, Jj" uniqKey="Mulvihill J">JJ Mulvihill</name>
</author>
</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Cerri, A" uniqKey="Cerri A">A Cerri</name>
</author>
<author><name sortKey="Gianni, C" uniqKey="Gianni C">C Gianni</name>
</author>
<author><name sortKey="Corbellino, M" uniqKey="Corbellino M">M Corbellino</name>
</author>
<author><name sortKey="Pizzuto, M" uniqKey="Pizzuto M">M Pizzuto</name>
</author>
<author><name sortKey="Moneghini, L" uniqKey="Moneghini L">L Moneghini</name>
</author>
<author><name sortKey="Crosti, C" uniqKey="Crosti C">C Crosti</name>
</author>
</analytic>
</biblStruct>
</listBibl>
</div1>
</back>
</TEI>
<pmc article-type="research-article"><pmc-dir>properties open_access</pmc-dir>
<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>
</contrib>
</contrib-group>
<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>
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Sante/explor/LymphedemaV1/Data/Pmc/Curation
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 003D10 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Pmc/Curation/biblio.hfd -nk 003D10 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien |wiki= Wicri/Sante |area= LymphedemaV1 |flux= Pmc |étape= Curation |type= RBID |clé= PMC:3776547 |texte= Cutaneous Epithelioid Clear Cells Angiosarcoma in a Young Woman with Congenital Lymphedema }}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/Pmc/Curation/RBID.i -Sk "pubmed:24078891" \ | HfdSelect -Kh $EXPLOR_AREA/Data/Pmc/Curation/biblio.hfd \ | NlmPubMed2Wicri -a LymphedemaV1
![]() | This area was generated with Dilib version V0.6.31. | ![]() |