Dermatofibrosarcoma protuberans is a rare, locally aggressive, rarely metastatic skin tumor which tends to recur. A 45-year-old female presented with dermatofibrosarcoma protuberans on her breast as a recurrent nodule after excision. Lump was re-excised with wide margins and irradiated. Follow-up was done monthly for two years, with no recurrence.
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Dermatofibrosarcoma protuberans (DFSP) is a rare, low to intermediate-grade soft tissue sarcoma that presents significant diagnostic and therapeutic challenges. We report the case of a 40-year-old male patient who presented with a slow-growing, asymptomatic lesion on his forehead that had developed over two years. Clinical examination revealed a firm, non-tender multinodular mass measuring 5 x 3 cm in the supraorbital region.
View Article and Find Full Text PDFHigh-grade sarcoma arising within a cellular dermatofibroma.
JAAD Case Rep
February 2025
Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
Fibrosarcomatous Dermatofibrosarcoma Protuberans With COL1A1-PDGFB Fusion in a 2-Year-Old Child: A Rare Occurrence With Spectrum of Histopathological Findings and Review of Literature.
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 PDFDermatofibrosarcoma Protuberans, Version 1.2025, NCCN Clinical Practice Guidelines In Oncology.
J 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 PDFDermatofibrosarcoma Protuberans: The Impact of the Surgical Incision Site in Relation to Tumor Recurrence.
Cureus
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.
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