A juvenile male case of dermatofibrosarcoma protuberans on the breast

Murase, Yuya; Takeichi, Takuya; Matsumoto, Takatoshi; Sakakibara, Ayako; Akiyama, Masashi

doi:10.1111/ced.13938

Cited by 5 publications

(8 citation statements)

References 6 publications

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“…15 This hypothesis is supported by genetic findings such as chromosomal translocation of t(17; 22) (q22; q13) between chromosomes 17 and 22, the fusion of gene COL6A3-PDGFD, and the fusion of gene COL1A1-PDGFB. 15,17…”

Section: Discussionmentioning

confidence: 99%

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

Bouhani

Fertani

Zemni

et al. 2019

Journal of Investigative Medicine High Impact Case Reports

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Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma of soft tissue representing about 1% of all tumors. In addition, DFSP occurs commonly on the trunk and extremities, and only a few cases of DFSP have been observed on the breast. In men, only 11 cases, including this case, have been reported. In this article, we present a case of left breast DFSP that occurred in a 44-year-old man. The physical examination revealed a left breast tender mass, which invaded the skin. The tumor was staged as T4b N0 M0. Mammography and sonography showed a suspect mass of the left breast. The biopsy and immunochemistry permitted the diagnosis of DFSP of the left breast. The patient had a left mammectomy, with free margins. He presents no evident sign of recurrence 7 months later.

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Section: Discussionmentioning

confidence: 99%

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

Bouhani

Fertani

Zemni

et al. 2019

Journal of Investigative Medicine High Impact Case Reports

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“…When difficult to diagnose, FISH analysis of the COL1A1-PDGF β fusion gene using routine biopsy specimens is a quick and convenient method [ 37 ]. In reported 5 cases of breast DFSP with gene detection, 4 cases presented the COL1A1-PDGF β fusion gene [ 9 , 23 , 38 , 39 ], another presented the PDGF β gene rearrangement [ 40 ].…”

Section: Discussionmentioning

confidence: 99%

“…It occurs most frequently between the second to fifth decades of life and typically appears in the dermis and subcutis [ 6 , 7 ]. DFSP can be all over the body; the most common site is the trunk (42-72%), followed by the proximal extremities (16-30%) [ 6 , 8 ], and breast involvement is uncommon [ 8 , 9 ]. Due to the rarity of breast DFSP cases, the current understanding of DFSP of the breast is still inadequate.…”

Section: Introductionmentioning

confidence: 99%

A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

Wang

Chen

et al. 2020

BioMed Research International

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Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade fibroblastic mesenchymal tumor derived from the dermis. The aim of this retrospective analysis was to summarize the clinicopathological data from our cases and published cases to offer more evidence for the recognition of dermatofibrosarcoma protuberans (DFSP). A total of 6 breast DFSP patients who had received treatment in our hospital were retrospectively enrolled, and detailed clinicopathological data were gathered for analysis. The median age was 29.5 years (ranging from 17 to 42 years). Most cases presented a red or brown-red, mobile, well-circumscribed, protruding, breast mass (ranging from 1 to 3 cm). For histopathology, all cases (6/6) showed a storiform pattern of spindle cells that were positive for CD34 (6/6) and Vimentin (5/6) and negative for smooth muscle actin (0/6) and S-100 protein (0/6). The majority of patients (5/6) underwent wide local excision, with 2 cases treated with radiotherapy. With a median follow-up of 36 months, all 6 patients survived without recurrence or metastasis. The PubMed database was used to search for similar cases. Eventually, 36 cases were included in this review, while cases without detailed clinical information or not reported in English were excluded from the analysis. To summarize, DFSP of the breast is an extremely rare malignancy characterized by spindle tumor cells arranged in a storiform pattern and positivity for CD34. The core needle biopsy is one of the crucial methods for its preoperative diagnosis. Management of DFSP is mainly based on surgical excision. It is prone to local recurrence, so long-term follow-up is required.

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“…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: 91%

“…Dermatofibrosarcoma protuberans (DFSP) is an uncommon mesenchymal tumor that accounts for approximately 0.1% of all malignancies (1) and represents less than 5% of all soft tissue sarcomas occurring in adults aged 30 to 40 years (2). The overall incidence is five cases in every 1 million persons annually (3).…”

Section: Introductionmentioning

confidence: 99%

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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A juvenile male case of dermatofibrosarcoma protuberans on the breast

Abstract: A memorable patient ª

Cited by 5 publications

References 6 publications

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

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

A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

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

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