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Dermatofibrosarcoma protuberans challenges: a case series and review of the literature 

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare variant of skin sarcoma which is characterized by proliferation of spindle cells in a storiform pattern. Although it is mostly benign in its primary stages, it can cause a high burden of morbidity unless it is thoroughly excised. CASE PRES...

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Autores principales: Sheidaei, Somayeh, Salehi, Mahsa, Abedian kenari, Fatemeh, Jafari, Hamid Reza
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9850584/
https://www.ncbi.nlm.nih.gov/pubmed/36653860
http://dx.doi.org/10.1186/s13256-022-03728-6
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author Sheidaei, Somayeh
Salehi, Mahsa
Abedian kenari, Fatemeh
Jafari, Hamid Reza
author_facet Sheidaei, Somayeh
Salehi, Mahsa
Abedian kenari, Fatemeh
Jafari, Hamid Reza
author_sort Sheidaei, Somayeh
collection PubMed
description BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare variant of skin sarcoma which is characterized by proliferation of spindle cells in a storiform pattern. Although it is mostly benign in its primary stages, it can cause a high burden of morbidity unless it is thoroughly excised. CASE PRESENTATION: Here, we review six cases of DFSP which were characterized by skin lesions in various parts of the body. Patients were from 26 to 51 years old; four were Asian men and two were Asian women. Wide surgical excision was performed for all these patients and no extra treatment was considered. Samples were studied by hematoxylin and eosin (H&E) staining and immunohistochemical (IHC) tests. Only one of our patients experienced recurrence after the initial surgery. CONCLUSION: Determining the best surgical method is still a dilemma in the treatment of DFSP lesions. There are numerous studies to prove the efficacy of various surgical interventions. Although DFSP is not commonly known as a malignant skin lesion, delay in treatment will have a catastrophic impact on patients’ lives. Thus, applying an in-time surgical method (wide local excision in our cases) in treating DFSP is crucial in preventing recurrence as well as decreasing the morbidity burden of DFSP.
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spelling pubmed-98505842023-01-20 Dermatofibrosarcoma protuberans challenges: a case series and review of the literature  Sheidaei, Somayeh Salehi, Mahsa Abedian kenari, Fatemeh Jafari, Hamid Reza J Med Case Rep Case Report BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare variant of skin sarcoma which is characterized by proliferation of spindle cells in a storiform pattern. Although it is mostly benign in its primary stages, it can cause a high burden of morbidity unless it is thoroughly excised. CASE PRESENTATION: Here, we review six cases of DFSP which were characterized by skin lesions in various parts of the body. Patients were from 26 to 51 years old; four were Asian men and two were Asian women. Wide surgical excision was performed for all these patients and no extra treatment was considered. Samples were studied by hematoxylin and eosin (H&E) staining and immunohistochemical (IHC) tests. Only one of our patients experienced recurrence after the initial surgery. CONCLUSION: Determining the best surgical method is still a dilemma in the treatment of DFSP lesions. There are numerous studies to prove the efficacy of various surgical interventions. Although DFSP is not commonly known as a malignant skin lesion, delay in treatment will have a catastrophic impact on patients’ lives. Thus, applying an in-time surgical method (wide local excision in our cases) in treating DFSP is crucial in preventing recurrence as well as decreasing the morbidity burden of DFSP. BioMed Central 2023-01-19 /pmc/articles/PMC9850584/ /pubmed/36653860 http://dx.doi.org/10.1186/s13256-022-03728-6 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Sheidaei, Somayeh
Salehi, Mahsa
Abedian kenari, Fatemeh
Jafari, Hamid Reza
Dermatofibrosarcoma protuberans challenges: a case series and review of the literature 
title Dermatofibrosarcoma protuberans challenges: a case series and review of the literature 
title_full Dermatofibrosarcoma protuberans challenges: a case series and review of the literature 
title_fullStr Dermatofibrosarcoma protuberans challenges: a case series and review of the literature 
title_full_unstemmed Dermatofibrosarcoma protuberans challenges: a case series and review of the literature 
title_short Dermatofibrosarcoma protuberans challenges: a case series and review of the literature 
title_sort dermatofibrosarcoma protuberans challenges: a case series and review of the literature 
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9850584/
https://www.ncbi.nlm.nih.gov/pubmed/36653860
http://dx.doi.org/10.1186/s13256-022-03728-6
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