Cutaneous Epithelioid Clear Cells Angiosarcoma in a Young Woman with Congenital Lymphedema
Identifieur interne : 000172 ( France/Analysis ); précédent : 000171; suivant : 000173Cutaneous 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
Affiliations:
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PMC:3776547Le document en format XML
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last="De Pinieux">Gonzague De Pinieux</name><affiliation wicri:level="3"><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><placeName><region type="region" nuts="2">Centre-Val de Loire</region><region type="old region" nuts="2">Région Centre</region><settlement type="city">Tours</settlement></placeName></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 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Delalande F" first="Flore" last="Tabareau-Delalande">Flore Tabareau-Delalande</name></region><name sortKey="De Muret, Anne" sort="De Muret, Anne" uniqKey="De Muret A" first="Anne" last="De Muret">Anne De Muret</name><name sortKey="De Pinieux, Gonzague" sort="De Pinieux, Gonzague" uniqKey="De Pinieux G" first="Gonzague" last="De Pinieux">Gonzague De Pinieux</name><name sortKey="Decouvelaere, Anne Valerie" sort="Decouvelaere, Anne Valerie" uniqKey="Decouvelaere A" first="Anne-Valérie" last="Decouvelaere">Anne-Valérie Decouvelaere</name><name sortKey="Miquelestorena Standley, Elodie" sort="Miquelestorena Standley, Elodie" uniqKey="Miquelestorena Standley E" first="Elodie" last="Miquelestorena-Standley">Elodie Miquelestorena-Standley</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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