Diagnosis and Management of Dermatofibrosarcoma Protuberans in a 3-Year-Old Patient

Iyengar, Rajiv; Kiwanuka, Elizabeth; Mangray, Shamlal; Cruz, Antonio P.; Katarincic, Julia A.; Bhatt, Reena

doi:10.1016/j.jhsa.2017.04.009

Cited by 7 publications

(9 citation statements)

References 15 publications

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“…This malignant fibroblastic tumor is characterized by a specific chromosomal translocation t(17;22)(q22;q13) in 90% of cases, which leads to fusion of the platelet-derived growth factor beta (PDGFbeta) gene with the collagen type 1 alpha 1 (COL1alpha1) gene [ 2 - 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…Morphologically, in a differential diagnosis, DFSP could be mistaken for dermatofibroma, peripheral nerve sheath tumors (e.g., neurofibroma or schwannoma), malignant melanoma, and morpheaform basal cell carcinoma [ 2 ]. It should be also noted that apart from DFSP, other tumors, such as epithelioid sarcoma, spindle cell lipoma, CD34-positive cellular digital fibromas, and solitary fibrous tumors, could also express CD34 [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

“…DFSP is rarely encountered in children and elderly persons. Several cases of congenital variants of DFSP have also been described [ 2 ]. Most commonly DFSP affects the trunk (40%-50%), followed by the proximal segments of the upper and lower limbs (30%-40%) and the head and neck region (10%-15%) [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

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A Rare Case of Dermatofibrosarcoma Protuberans of the Thumb in an 80-year-old Woman

Georgiev

Slavchev

Ananiev

2018

Cureus

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Dermatofibrosarcoma protuberans (DFSP) has been described as a rare, locally invasive, malignant fibroblastic tumor with a high rate of recurrence that usually affects middle-aged patients. Herein, we describe a rare case of a pedunculated dermatofibrosarcoma protuberans of the right thumb in an 80-year-old woman treated with excision through the base of the pedicle. We also make a brief literature review concerning this tumor.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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A Rare Case of Dermatofibrosarcoma Protuberans of the Thumb in an 80-year-old Woman

Georgiev

Slavchev

Ananiev

2018

Cureus

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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: 92%

“…It is a locally aggressive fibrous tumor, with a high recurrence rate (3). Therefore, it is important to achieve negative margins to minimize disease recurrence (5). DFSP can give rise to distant metastases, usually to the bones and lungs (incidence is less than 5%) (6).…”

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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Giant Cell Fibroblastoma/Dermatofibrosarcoma Protuberans

2018

Diagnostic Pathology: Pediatric Neoplasms

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Diagnosis and Management of Dermatofibrosarcoma Protuberans in a 3-Year-Old Patient

Cited by 7 publications

References 15 publications

A Rare Case of Dermatofibrosarcoma Protuberans of the Thumb in an 80-year-old Woman

A Rare Case of Dermatofibrosarcoma Protuberans of the Thumb in an 80-year-old Woman

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

Giant Cell Fibroblastoma/Dermatofibrosarcoma Protuberans

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