Vulvar Darier-Ferrand dermatofibrosarcoma: unusual localization of a rare tumor.
Dermatofibrosarcoma
histopathology
surgery
vulva
Journal
The Pan African medical journal
ISSN: 1937-8688
Titre abrégé: Pan Afr Med J
Pays: Uganda
ID NLM: 101517926
Informations de publication
Date de publication:
2019
2019
Historique:
received:
17
04
2019
accepted:
02
05
2019
entrez:
27
8
2019
pubmed:
27
8
2019
medline:
27
9
2019
Statut:
epublish
Résumé
Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade sarcoma of dermal origin that rarely presents in the vulva, typically occurring on the trunk of young to middle-aged adults. Even though it has a low potential for distant metastases, it often recurs locally. Therefore, surgical excision is the treatment of choice. We report a case of DFSP with fibrosarcoma transformation involving the vulva treated by large excision with tumor free margins followed by plastic reconstruction. Our aim is to highlight this rare disease and through literature evaluate the safety and efficacy of surgical treatment. Early recognition of this rare entity whose localization and the aggressive nature of the fibrosarcomatous component will be an issue in the surgical management.
Identifiants
pubmed: 31448009
doi: 10.11604/pamj.2019.33.46.18861
pii: PAMJ-33-46
pmc: PMC6689845
doi:
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
46Déclaration de conflit d'intérêts
The authors declare no competing interests.
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