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

Abstract 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. C...

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Main Authors: Somayeh Sheidaei, Mahsa Salehi, Fatemeh Abedian kenari, Hamid Reza Jafari
Format: Article
Language:English
Published: BMC 2023-01-01
Series:Journal of Medical Case Reports
Subjects:
Online Access:https://doi.org/10.1186/s13256-022-03728-6
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author Somayeh Sheidaei
Mahsa Salehi
Fatemeh Abedian kenari
Hamid Reza Jafari
author_facet Somayeh Sheidaei
Mahsa Salehi
Fatemeh Abedian kenari
Hamid Reza Jafari
author_sort Somayeh Sheidaei
collection DOAJ
description Abstract 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 doaj.art-e36abbabc1654245b129b82517f7183a2023-01-22T12:14:51ZengBMCJournal of Medical Case Reports1752-19472023-01-011711810.1186/s13256-022-03728-6Dermatofibrosarcoma protuberans challenges: a case series and review of the literature Somayeh Sheidaei0Mahsa Salehi1Fatemeh Abedian kenari2Hamid Reza Jafari3Pathology, Mazandaran University of Medical SciencesPathology, Mazandaran University of Medical SciencesPathology, Mazandaran University of Medical SciencesGeneral surgery, Isfahan University of Medical SciencesAbstract 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.https://doi.org/10.1186/s13256-022-03728-6Dermatofibrosarcoma protuberans (DFSP)Wide local excision (WLE)
spellingShingle Somayeh Sheidaei
Mahsa Salehi
Fatemeh Abedian kenari
Hamid Reza Jafari
Dermatofibrosarcoma protuberans challenges: a case series and review of the literature 
Journal of Medical Case Reports
Dermatofibrosarcoma protuberans (DFSP)
Wide local excision (WLE)
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 Dermatofibrosarcoma protuberans (DFSP)
Wide local excision (WLE)
url https://doi.org/10.1186/s13256-022-03728-6
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AT mahsasalehi dermatofibrosarcomaprotuberanschallengesacaseseriesandreviewoftheliterature
AT fatemehabediankenari dermatofibrosarcomaprotuberanschallengesacaseseriesandreviewoftheliterature
AT hamidrezajafari dermatofibrosarcomaprotuberanschallengesacaseseriesandreviewoftheliterature