Forty-one cases of dermatofibrosarcoma protuberans are presented. The clinical features and histopathological appearances are described. Immunohistochemical staining of thirteen cases with antisera to lysozyme, alpha 1-antichymotrypsin and S-100 protein has provided no evidence to support either a histiocytic or neuroectodermal origin for these tumours. In reviewing the literature, the histogenetic origin, differential diagnosis and malignant potential of dermatofibrosarcoma protuberans are discussed.
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http://dx.doi.org/10.1111/j.1365-2559.1985.tb02878.x | DOI Listing |
JAAD Case Rep
February 2025
Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
Pediatr Dev Pathol
January 2025
Department of Medical Oncology, Artemis Hospital, Gurgaon, Haryana, India.
Dermatofibrosarcoma protuberans (DFSP) is an intermediate-grade fibroblastic neoplasm commonly seen in young and middle-aged patients and rarely in pediatric patients. Fibrosarcomatous transformation is common in adults but extremely uncommon in children. Here, we present a case of a 2-year-old child who presented with a progressively enlarging subcutaneous mass in the knee.
View Article and Find Full Text PDFJ Natl Compr Canc Netw
January 2025
National Comprehensive Cancer Network.
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue sarcoma and affects an estimated 1,500 people annually in the United States. DFSP frequently exhibits extensive local infiltration. Initial treatment is through surgical excision, and care should be taken to ensure that negative margins are achieved to minimize recurrence.
View Article and Find Full Text PDFCureus
December 2024
Pathology, Avalon University School of Medicine, Willemstad, CUW.
Dermatofibrosarcoma protuberans (DFSP) is a rare, locally invasive cutaneous sarcoma with a high propensity for recurrence, even following complete surgical excision. DFSP exhibits a low metastatic potential and is characterized by a distinctive honeycomb-like architecture composed of uniformly arranged spindle cells that frequently show CD34 immunostaining. Common surgical approaches include wide local excision (WLE), Mohs micrographic surgery (MMS), and, in severe cases, amputation.
View Article and Find Full Text PDFCancers (Basel)
January 2025
Unit of Dermatology, Department of Medicine, University of Padova, 35122 Padua, Italy.
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