A male case of dermatofibrosarcoma protuberans in the breast presenting as gynecomastia

Introduction. Dermatofibrosarcoma protuberans (DFSP) is a very rare mesenchymal tumor that accounts for approximately 0.1% of all malignancies. It is a locally aggressive fibrous tumor, with a high recurrence rate, which sometimes gives rise to distant metastases, usually to the bones and lungs. DFS...

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Autores principales:	Vučaj-Ćirilović Viktorija, Vuković Miloš, Boban Jasmina, Šolajić Nenad, Ranisavljević Milan, Prvulović-Bunović Nataša
Formato:	article
Lenguaje:	EN SR
Publicado:	Military Health Department, Ministry of Defance, Serbia 2021
Materias:	breast neoplasms, male dermatofibrosarcoma diagnosis, differential immunohistochemistry rare diseases Medicine (General) R5-920
Acceso en línea:	https://doaj.org/article/73c76dae161e47efb21deef1dc8b6910
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spelling	oai:doaj.org-article:73c76dae161e47efb21deef1dc8b69102021-11-10T07:27:58ZA male case of dermatofibrosarcoma protuberans in the breast presenting as gynecomastia0042-84502406-072010.2298/VSP191124042Vhttps://doaj.org/article/73c76dae161e47efb21deef1dc8b69102021-01-01T00:00:00Zhttp://www.doiserbia.nb.rs/img/doi/0042-8450/2021/0042-84502000042V.pdfhttps://doaj.org/toc/0042-8450https://doaj.org/toc/2406-0720Introduction. Dermatofibrosarcoma protuberans (DFSP) is a very rare mesenchymal tumor that accounts for approximately 0.1% of all malignancies. It is a locally aggressive fibrous tumor, with a high recurrence rate, which sometimes gives rise to distant metastases, usually to the bones and lungs. DFSP usually occurs on the trunk and extremities with only a small number of cases in the breast, especially in men. Case report. We presented a rare case of DFSP in the male breast. A 66-year-old man presented with gynecomastia of the left breast. The diagnostic work-up comprised of clinical examination, ultrasonography, core biopsy, and mammography. Immunohistochemistry revealed diffuse and strong positivity for vimentin, CD99, and CD34, while the tumor cells were completely negative for keratin, S100 protein, STAT6, CD31, and factor VIII, highly suggestive for DFSP. Subsequently, a radical mastectomy was performed and preoperative diagnosis of DESP was confirmed by pathological examination and immunohistochemistry. The patient was still disease-free six months after the surgical treatment. Conclusion. DFSP is a soft tissue sarcoma that rarely occurs in the breast, especially in men. The most common clinical presentation in the breast is a mass with extensive nodules on the surface, but it can also be presented as gynecomastia, as in our case. The diagnosis of DFPS is based on anatomopathology with immunohistochemistry analysis since there are no specific imaging features for this rare entity. Surgical excision with wide and negative margins is optimal for reducing the risk of recurrence.Vučaj-Ćirilović ViktorijaVuković MilošBoban JasminaŠolajić NenadRanisavljević MilanPrvulović-Bunović NatašaMilitary Health Department, Ministry of Defance, Serbiaarticlebreast neoplasms, maledermatofibrosarcomadiagnosis, differentialimmunohistochemistryrare diseasesMedicine (General)R5-920ENSRVojnosanitetski Pregled, Vol 78, Iss 10, Pp 1108-1112 (2021)
institution	DOAJ
collection	DOAJ
language	EN SR
topic	breast neoplasms, male dermatofibrosarcoma diagnosis, differential immunohistochemistry rare diseases Medicine (General) R5-920
spellingShingle	breast neoplasms, male dermatofibrosarcoma diagnosis, differential immunohistochemistry rare diseases Medicine (General) R5-920 Vučaj-Ćirilović Viktorija Vuković Miloš Boban Jasmina Šolajić Nenad Ranisavljević Milan Prvulović-Bunović Nataša A male case of dermatofibrosarcoma protuberans in the breast presenting as gynecomastia
description	Introduction. Dermatofibrosarcoma protuberans (DFSP) is a very rare mesenchymal tumor that accounts for approximately 0.1% of all malignancies. It is a locally aggressive fibrous tumor, with a high recurrence rate, which sometimes gives rise to distant metastases, usually to the bones and lungs. DFSP usually occurs on the trunk and extremities with only a small number of cases in the breast, especially in men. Case report. We presented a rare case of DFSP in the male breast. A 66-year-old man presented with gynecomastia of the left breast. The diagnostic work-up comprised of clinical examination, ultrasonography, core biopsy, and mammography. Immunohistochemistry revealed diffuse and strong positivity for vimentin, CD99, and CD34, while the tumor cells were completely negative for keratin, S100 protein, STAT6, CD31, and factor VIII, highly suggestive for DFSP. Subsequently, a radical mastectomy was performed and preoperative diagnosis of DESP was confirmed by pathological examination and immunohistochemistry. The patient was still disease-free six months after the surgical treatment. Conclusion. DFSP is a soft tissue sarcoma that rarely occurs in the breast, especially in men. The most common clinical presentation in the breast is a mass with extensive nodules on the surface, but it can also be presented as gynecomastia, as in our case. The diagnosis of DFPS is based on anatomopathology with immunohistochemistry analysis since there are no specific imaging features for this rare entity. Surgical excision with wide and negative margins is optimal for reducing the risk of recurrence.
format	article
author	Vučaj-Ćirilović Viktorija Vuković Miloš Boban Jasmina Šolajić Nenad Ranisavljević Milan Prvulović-Bunović Nataša
author_facet	Vučaj-Ćirilović Viktorija Vuković Miloš Boban Jasmina Šolajić Nenad Ranisavljević Milan Prvulović-Bunović Nataša
author_sort	Vučaj-Ćirilović Viktorija
title	A male case of dermatofibrosarcoma protuberans in the breast presenting as gynecomastia
title_short	A male case of dermatofibrosarcoma protuberans in the breast presenting as gynecomastia
title_full	A male case of dermatofibrosarcoma protuberans in the breast presenting as gynecomastia
title_fullStr	A male case of dermatofibrosarcoma protuberans in the breast presenting as gynecomastia
title_full_unstemmed	A male case of dermatofibrosarcoma protuberans in the breast presenting as gynecomastia
title_sort	male case of dermatofibrosarcoma protuberans in the breast presenting as gynecomastia
publisher	Military Health Department, Ministry of Defance, Serbia
publishDate	2021
url	https://doaj.org/article/73c76dae161e47efb21deef1dc8b6910
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A male case of dermatofibrosarcoma protuberans in the breast presenting as gynecomastia

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