LymphedemaV1, PubMed, Corpus, bibRecord, 001902

Cutaneous epithelioid clear cells angiosarcoma in a young woman with congenital lymphedema.

Identifieur interne : 001902 ( PubMed/Corpus ); précédent : 001901; suivant : 001903

Cutaneous epithelioid clear cells angiosarcoma in a young woman with congenital lymphedema.

Auteurs : Flore Tabareau-Delalande ; Anne De Muret ; Elodie Miquelestorena-Standley ; Anne-Valérie Decouvelaere ; Gonzague De Pinieux

Source :

Case reports in pathology [ 2090-6781 ] ; 2013.

RBID : pubmed:24078891

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 en bloc 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 c-MYC amplification status was assessed. The diagnostic value of this amplification should be further evaluated in this specific context.

DOI: 10.1155/2013/931973
PubMed: 24078891

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<author><name sortKey="Tabareau Delalande, Flore" sort="Tabareau Delalande, Flore" uniqKey="Tabareau Delalande F" first="Flore" last="Tabareau-Delalande">Flore Tabareau-Delalande</name>
<affiliation><nlm:affiliation>Department of Pathology, Tours University Hospital, 37044 Tours, France.</nlm:affiliation>
</affiliation>
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<author><name sortKey="De Muret, Anne" sort="De Muret, Anne" uniqKey="De Muret A" first="Anne" last="De Muret">Anne De Muret</name>
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<author><name sortKey="Miquelestorena Standley, Elodie" sort="Miquelestorena Standley, Elodie" uniqKey="Miquelestorena Standley E" first="Elodie" last="Miquelestorena-Standley">Elodie Miquelestorena-Standley</name>
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<author><name sortKey="Decouvelaere, Anne Valerie" sort="Decouvelaere, Anne Valerie" uniqKey="Decouvelaere A" first="Anne-Valérie" last="Decouvelaere">Anne-Valérie Decouvelaere</name>
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<author><name sortKey="De Pinieux, Gonzague" sort="De Pinieux, Gonzague" uniqKey="De Pinieux G" first="Gonzague" last="De Pinieux">Gonzague De Pinieux</name>
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<front><div type="abstract" xml:lang="en">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 en bloc 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 c-MYC amplification status was assessed. The diagnostic value of this amplification should be further evaluated in this specific context.</div>
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Cutaneous epithelioid clear cells angiosarcoma in a young woman with congenital lymphedema.

Cutaneous epithelioid clear cells angiosarcoma in a young woman with congenital lymphedema.

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Abstract

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