Dermatofibrosarcoma Protuberans of the Breast in Man: An Extremely Rare Entity With a Review of the Literature

Bouhani, Malek; Fertani, Y; Zemni, Inès; Adouni, Olfa; Bouida, A; Chargui, Riadh; Rahal, Khaled

doi:10.1177/2324709619875634

Cited by 8 publications

(14 citation statements)

References 16 publications

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“…The distribution of DFSP between genders shows that women have higher incidence rates than men except among the elderly (15), which is in opposition to statements of other reports in which men are slightly more commonly affected than women (9). In our case, the tumor on the male breast appeared much later, at the age of 66, compared to the recently published review of Bouhani et al (7) who state that the mean age of DFSP in the male breast is 32.6 years. The tumor is usually less than 5 cm in size, similarly to our case.…”

Section: Case Reportcontrasting

confidence: 99%

“…Immunohistochemically, DFSP usually shows strong positivity for CD34 (in 97% of the patients (1)), vimentin and negative staining for cytokeratin, S-100, epithelial membrane antigen and variable staining for smooth muscle actin (SMA) (12). The present case showed positivity for CD34 along with negativity for S-100 and only focal positivity for SMA, similar to the most recent reported DFSP case in the male breast (7).…”

Section: Case Reportsupporting

confidence: 83%

“…It remains unclear if higher estrogen levels or disbalance with testosterone levels can be a potential risk factor for developing this kind of malignancy. Regarding the recently published literature review (7), this case represents the 12 th case of the breast DFSP in men, which shows the rarity of this entity.…”

Section: Case Reportmentioning

confidence: 78%

“…DFSP usually occurs on the trunk and extremities with only a small number of cases in the breast, especially in men (1,2,5,7,12,13,14). We report a rare case of DFSP in the male breast that clinically presented as gynecomastia.…”

mentioning

confidence: 92%

“…The most common presentation in a male's breast is a mass with extensive nodules on the surface (7). The tumor tends to spare adnexal structures and is commonly superficially located, but in recurrent cases and untreated tumors, it can spread to more deeply situated structures (8,9).…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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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. This paper analyzes 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 dermatofibrosarcoma protuberans 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, but it can also present 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. Keywords: dermatofibrosarcoma protuberans; male breast; rare; CD34; STAT6; immunohistochemistry Apstrakt Uvod. Dermatofibrosarkom protuberans (DFSP) je veoma redak mezenhimalni tumor koji čini otprilike 0,1% svih maligniteta. To je lokalno agresivni fibrozni tumor, sa velikom stopom recidiva, koji ponekad može dati udaljene metastaze, obično u kosti i pluća. DFSP se obično javlja na trupu i ekstremitetima, dok se samo mali broj slučajeva javlja u dojci, posebno kod muškaraca. Prikaz bolesnika. Ovaj rad analizira retki slučaj DFSP u muškoj dojci. 66-godišnji muškarac je javio sa ginekomastijom leve dojke. Dijagnostička obrada sastojala se od kliničkog pregleda, ultrazvuka, kor biopsije i mamografije. Imunohistohemija je pokazala difuznu i jaku pozitivnost za vimentin, CD99 i CD34, dok su tumorske ćelije bile potpuno negativne na keratin, S100 protein, STAT6, CD31 i faktor VIII, što je sugerisalo da 4 se radi o DFSP. Nakon toga izvršena je radikalna mastektomija i patološkim pregledom i imunohistohemijom dokazana je preoperativna dijagnoza dermatofibrosarkoma protuberans. Pacijent je šest meseci nakon hirurškog lečenja i dalje bio bez bolesti. Zaključak. DFSP je sarkom mekog tkiva koji se izuzetno retko razvija u dojkama muškaraca. Uobičajena klinička prezentacija na dojci jeste masa sa širokim krvžicama na površini, mada se može prezentovati i samo kao ginekomastija, kao u našem slučaju. Dijagnoza DFPS se zasniva na patološkoj anatomiji sa imunoh...

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Section: Case Reportcontrasting

confidence: 99%

Section: Case Reportsupporting

confidence: 83%

Section: Case Reportmentioning

confidence: 78%

mentioning

confidence: 92%

Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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DFSP of the Breast: Histomorphological, Immunohistochemical, and Molecular Features of a Rare Case in an Unusual Location

Laharwani

Prakash²,

Walley³

et al. 2021

Applied Immunohistochemistry &Amp; Molecular Morphology

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We present a case of a 21-year-old female with a vague nontender mass in the lower inner quadrant of the left breast discovered incidentally on chest imaging following trauma. A breast ultrasound demonstrated an 8×6×8 mm irregular hyperechoic mass at the 7 o'clock position of the left breast, 9 cm from the nipple. The mass was graded Breast Imaging Reporting and Data System (BI-RADS) category 4 (suspicious finding). An ultrasound-guided biopsy of the mass showed a proliferation of monotonous spindled cells in a storiform pattern with tapered nuclei with infiltration into the adipose tissue. No normal breast elements were identified in the biopsy. Myofibroblastoma was the first differential diagnosis; however, the characteristic infiltrative pattern of the tumor mandated additional tests including fluorescence in situ hybridization to rule out a dermatofibrosarcoma protruberance (DFSP). Immunohistochemical staining showed positive staining for CD34, which can be positive in myofibroblastoma also. However, fluorescence in situ hybridization demonstrated a platelet-derived growth factor B (22q13.1) gene rearrangement confirming a diagnosis of DFSP. The patient underwent a wide local excision of the DFSP for definitive treatment. She is doing well with no recurrence reported so far, after 15 months of follow-up. Conventional DFSP does not metastasize but is prone to recurrence making wide margins imperative for definitive treatment.

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Dermatofibrosarcoma Protuberans Mimicking Primary Breast Neoplasm: A case report and literature review

Al-Farsi

Al-Brashdi

Al-Salhi

et al. 2020

Sultan Qaboos Univ Med J

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Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing mesenchymal neoplasm of the dermis and subcutaneous tissues that has a low- to intermediate-grade malignancy. DFSP commonly involves the trunk and extremities, and very rarely the breast skin, mimicking a primary breast neoplasm with few reported cases in the literature. We report a 35-year old female patient who was referred to the Royal Hospital, Muscat, Oman in 2017, with a two-year history of a slow growing left breast lump. Assessment of the breasts with mammography revealed a lobulated lesion in the left-upper-inner quadrant with neither microcalcification nor architectural distortion, mimicking a benign lesion. However, on ultrasound, the lesion had suspicious features with increased vascularity and hence, it was categorised as breast imaging reporting and data system (BIRAD) IV. The patient underwent left breast wide local excision and the histopathological diagnosis was dermatofibrosarcoma protuberans.Keywords: Breast; Dermatofibrosarcoma Protuberans; Ultrasonography; Oman.

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Dermatofibrosarcoma Protuberans of the Breast in Man: An Extremely Rare Entity With a Review of the Literature

Cited by 8 publications

References 16 publications

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

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

DFSP of the Breast: Histomorphological, Immunohistochemical, and Molecular Features of a Rare Case in an Unusual Location

Dermatofibrosarcoma Protuberans Mimicking Primary Breast Neoplasm: A case report and literature review

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