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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Ministry of Defence of the Republic of Serbia, University of Defence, Belgrade
2021-01-01
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| Series: | Vojnosanitetski Pregled |
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| Online Access: | http://www.doiserbia.nb.rs/img/doi/0042-8450/2021/0042-84502000042V.pdf |
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| 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 |
| collection | DOAJ |
| 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 |
| id | doaj-art-73c76dae161e47efb21deef1dc8b6910 |
| institution | OA Journals |
| issn | 0042-8450 2406-0720 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Ministry of Defence of the Republic of Serbia, University of Defence, Belgrade |
| record_format | Article |
| series | Vojnosanitetski Pregled |
| spelling | doaj-art-73c76dae161e47efb21deef1dc8b69102025-08-20T02:07:05ZengMinistry of Defence of the Republic of Serbia, University of Defence, BelgradeVojnosanitetski Pregled0042-84502406-07202021-01-0178101108111210.2298/VSP191124042V0042-84502000042VA male case of dermatofibrosarcoma protuberans in the breast presenting as gynecomastiaVučaj-Ćirilović Viktorija0Vuković Miloš1Boban Jasmina2https://orcid.org/0000-0001-9701-5484Šolajić Nenad3https://orcid.org/0000-0002-0507-2682Ranisavljević Milan4Prvulović-Bunović Nataša5https://orcid.org/0000-0003-2303-5874University of Novi Sad, Faculty of Medicine, Department of Radiology, Novi Sad, Serbia + Oncology Institute of Vojvodina, Center for Imiging Diagnostics, Novi Sad, SerbiaUniversity of Novi Sad, Faculty of Medicine, Department of Radiology, Novi Sad, Serbia + Oncology Institute of Vojvodina, Center for Imiging Diagnostics, Novi Sad, SerbiaUniversity of Novi Sad, Faculty of Medicine, Department of Radiology, Novi Sad, Serbia + Oncology Institute of Vojvodina, Center for Imiging Diagnostics, Novi Sad, SerbiaUniversity of Novi Sad, Faculty of Medicine, Department of Pathology, Novi Sad, Serbia + Oncology Institute of Vojvodina, Department of Pathoanatomical and Laboratory Diagnostics, Novi Sad, SerbiaUniversity of Novi Sad, Faculty of Medicine, Department of Surgery, Novi Sad, Serbia + Oncology Institute of Vojvodina, Clinic for Oncological Surgery, Novi Sad, SerbiaUniversity of Novi Sad, Faculty of Medicine, Department of Radiology, Novi Sad, Serbia + Oncology Institute of Vojvodina, Center for Imiging Diagnostics, Novi Sad, SerbiaIntroduction. 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.http://www.doiserbia.nb.rs/img/doi/0042-8450/2021/0042-84502000042V.pdfbreast neoplasms, maledermatofibrosarcomadiagnosis, differentialimmunohistochemistryrare diseases |
| spellingShingle | 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 Vojnosanitetski Pregled breast neoplasms, male dermatofibrosarcoma diagnosis, differential immunohistochemistry rare diseases |
| title | 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_short | 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 |
| topic | breast neoplasms, male dermatofibrosarcoma diagnosis, differential immunohistochemistry rare diseases |
| url | http://www.doiserbia.nb.rs/img/doi/0042-8450/2021/0042-84502000042V.pdf |
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