Dermatofibrosarcoma protuberans (DFSP) is a rare locally aggressive tumor with distant metastases being unusual. We present a case of metastatic DFSP treated with imatinib showing complete metabolic response to treatment.
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http://dx.doi.org/10.4103/0972-3919.181528 | DOI Listing |
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 PDFJAAD 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.
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