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Cutaneous Angiosarcoma in an Unusual Location and Without Predisposing Factors

Cutaneous angiosarcoma is a rare, highly malignant tumour of vascular endothelial origin. It usually arises in the skin and superficial soft tissue, mostly on the head and neck. It presents as a variety of lesions, and so is considered a great mimicker, leading to a delay in diagnosis and evidencing...

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Autores principales: Oliveira Monteiro, Ana, Branco Ferreira, Inês, César, Artur, Mota, Alberto, Pinheiro, Jorge, Lopes, José Manuel, Cardoso, M Teresa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SMC Media Srl 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7727643/
https://www.ncbi.nlm.nih.gov/pubmed/33313005
http://dx.doi.org/10.12890/2020_001939
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author Oliveira Monteiro, Ana
Branco Ferreira, Inês
César, Artur
Mota, Alberto
Pinheiro, Jorge
Lopes, José Manuel
Cardoso, M Teresa
author_facet Oliveira Monteiro, Ana
Branco Ferreira, Inês
César, Artur
Mota, Alberto
Pinheiro, Jorge
Lopes, José Manuel
Cardoso, M Teresa
author_sort Oliveira Monteiro, Ana
collection PubMed
description Cutaneous angiosarcoma is a rare, highly malignant tumour of vascular endothelial origin. It usually arises in the skin and superficial soft tissue, mostly on the head and neck. It presents as a variety of lesions, and so is considered a great mimicker, leading to a delay in diagnosis and evidencing the importance of biopsy with immunohistochemistry confirmation. There are few reports of extremity involvement in patients with pre-existing chronic lymphoedema, or exposure to radiation therapy. We report the case of an 82-year-old woman with lower limb extensive cutaneous involvement, distant metastatic disease, and poor therapy response. Its rare location without predisposing factors highlights the need to raise awareness about this disease. LEARNING POINTS: Extremity involvement of cutaneous angiosarcoma has been rarely described. The marked heterogeneity in presentation leads to a delay in diagnosis and poor prognosis, so the index of suspicion should be high. The cases reported in the literature describe a well-known relationship between cutaneous angiosarcoma and predisposing factors, but its absence should not exclude the diagnosis. This case highlights the importance of recognizing and biopsy suspected skin lesions for immunohistochemistry diagnostic confirmation.
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spelling pubmed-77276432020-12-11 Cutaneous Angiosarcoma in an Unusual Location and Without Predisposing Factors Oliveira Monteiro, Ana Branco Ferreira, Inês César, Artur Mota, Alberto Pinheiro, Jorge Lopes, José Manuel Cardoso, M Teresa Eur J Case Rep Intern Med Articles Cutaneous angiosarcoma is a rare, highly malignant tumour of vascular endothelial origin. It usually arises in the skin and superficial soft tissue, mostly on the head and neck. It presents as a variety of lesions, and so is considered a great mimicker, leading to a delay in diagnosis and evidencing the importance of biopsy with immunohistochemistry confirmation. There are few reports of extremity involvement in patients with pre-existing chronic lymphoedema, or exposure to radiation therapy. We report the case of an 82-year-old woman with lower limb extensive cutaneous involvement, distant metastatic disease, and poor therapy response. Its rare location without predisposing factors highlights the need to raise awareness about this disease. LEARNING POINTS: Extremity involvement of cutaneous angiosarcoma has been rarely described. The marked heterogeneity in presentation leads to a delay in diagnosis and poor prognosis, so the index of suspicion should be high. The cases reported in the literature describe a well-known relationship between cutaneous angiosarcoma and predisposing factors, but its absence should not exclude the diagnosis. This case highlights the importance of recognizing and biopsy suspected skin lesions for immunohistochemistry diagnostic confirmation. SMC Media Srl 2020-09-25 /pmc/articles/PMC7727643/ /pubmed/33313005 http://dx.doi.org/10.12890/2020_001939 Text en © EFIM 2020 This article is licensed under a Commons Attribution Non-Commercial 4.0 License (https://creativecommons.org/licenses/by-nc-nd/4.0/)
spellingShingle Articles
Oliveira Monteiro, Ana
Branco Ferreira, Inês
César, Artur
Mota, Alberto
Pinheiro, Jorge
Lopes, José Manuel
Cardoso, M Teresa
Cutaneous Angiosarcoma in an Unusual Location and Without Predisposing Factors
title Cutaneous Angiosarcoma in an Unusual Location and Without Predisposing Factors
title_full Cutaneous Angiosarcoma in an Unusual Location and Without Predisposing Factors
title_fullStr Cutaneous Angiosarcoma in an Unusual Location and Without Predisposing Factors
title_full_unstemmed Cutaneous Angiosarcoma in an Unusual Location and Without Predisposing Factors
title_short Cutaneous Angiosarcoma in an Unusual Location and Without Predisposing Factors
title_sort cutaneous angiosarcoma in an unusual location and without predisposing factors
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7727643/
https://www.ncbi.nlm.nih.gov/pubmed/33313005
http://dx.doi.org/10.12890/2020_001939
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