Vulvar Dermatofibrosarcoma protuberance in Iran and a narrative review of literature: A first case report.
Dermatofibrosarcoma Protuberan
Fibrosarcomatous
Neoplasia
Vulva
Journal
Caspian journal of internal medicine
ISSN: 2008-6164
Titre abrégé: Caspian J Intern Med
Pays: Iran
ID NLM: 101523876
Informations de publication
Date de publication:
2023
2023
Historique:
medline:
31
7
2023
pubmed:
31
7
2023
entrez:
31
7
2023
Statut:
ppublish
Résumé
Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor that originates from deep layers of the dermis and mainly is common in young adults to middle age. This tumor is rare in the vulva. A 53-year-old multiparous menopause female had complained of asymptomatic swelling of the right labia major. Dermatofibrosarcoma protuberans was reported in primary tumor resection. Six months later, rapid growing mass recurred in the vulva. The patient underwent radical Vulvectomy and resection of the margin of about 2-3 cm along with bilateral Inguinofemoral lymphadenectomy. The margins of the mass were negative in the frozen section. Microscopic examination revealed that hypercellular neoplasm in dermis comprising monomorphic spindle cells with high mitotic activity, some hyperchromatic nuclei arranged in palisading fashion. Microscopic and IHC study confirmed the conversion of dermatofibrosarcoma protuberans to fibrosarcoma. This case was presented due to the rarity of dermatofibrosarcoma protuberance in the vulva and pathologic conversion to fibrosarcoma.
Sections du résumé
Background
UNASSIGNED
Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor that originates from deep layers of the dermis and mainly is common in young adults to middle age. This tumor is rare in the vulva.
Case Presentation
UNASSIGNED
A 53-year-old multiparous menopause female had complained of asymptomatic swelling of the right labia major. Dermatofibrosarcoma protuberans was reported in primary tumor resection. Six months later, rapid growing mass recurred in the vulva. The patient underwent radical Vulvectomy and resection of the margin of about 2-3 cm along with bilateral Inguinofemoral lymphadenectomy. The margins of the mass were negative in the frozen section. Microscopic examination revealed that hypercellular neoplasm in dermis comprising monomorphic spindle cells with high mitotic activity, some hyperchromatic nuclei arranged in palisading fashion. Microscopic and IHC study confirmed the conversion of dermatofibrosarcoma protuberans to fibrosarcoma.
Conclusion
UNASSIGNED
This case was presented due to the rarity of dermatofibrosarcoma protuberance in the vulva and pathologic conversion to fibrosarcoma.
Identifiants
pubmed: 37520865
doi: 10.22088/cjim.14.3.572
pmc: PMC10379794
doi:
Types de publication
Case Reports
Langues
eng
Pagination
572-576Références
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