Academic Journal
Cutaneous Epithelioid Clear Cells Angiosarcoma in a Young Woman with Congenital Lymphedema
العنوان: | Cutaneous Epithelioid Clear Cells Angiosarcoma in a Young Woman with Congenital Lymphedema |
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المؤلفون: | Flore Tabareau-Delalande, Anne de Muret, Elodie Miquelestorena-Standley, Anne-Valérie Decouvelaere, Gonzague de Pinieux |
المصدر: | Case Reports in Pathology, Vol 2013 (2013) |
بيانات النشر: | Hindawi Limited, 2013. |
سنة النشر: | 2013 |
المجموعة: | LCC:Pathology |
مصطلحات موضوعية: | Pathology, RB1-214 |
الوصف: | 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. |
نوع الوثيقة: | article |
وصف الملف: | electronic resource |
اللغة: | English |
تدمد: | 2090-6781 2090-679X |
Relation: | https://doaj.org/toc/2090-6781; https://doaj.org/toc/2090-679X |
DOI: | 10.1155/2013/931973 |
URL الوصول: | https://doaj.org/article/b6ef802c5eed489b821db44d4d064b79 |
رقم الانضمام: | edsdoj.b6ef802c5eed489b821db44d4d064b79 |
قاعدة البيانات: | Directory of Open Access Journals |
تدمد: | 20906781 2090679X |
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DOI: | 10.1155/2013/931973 |