Dermatofibrosarcoma protuberans (DFSP) is a rare malignant neoplasm of skin, subcutaneous tissue and dermis, with more potential of local infiltration, appears most frequently on the trunk, followed by extremities, head, neck, and extremely rare in breast. It often occurs in young adults and middle-aged adults. The standard of treatment for this disease is local surgical excision with 2-3 cm wide margins. We present a case of 49-year lady with right breast well-circumscribed, firm, tender, mobile, and progressive lump that was benign-looking, on physical examination as well as on mammography. She underwent local surgical resection and diagnosed as DFSP on microscopic and immunohistochemical analysis with positive deep margin. She was referred for further multidisciplinary management. Case was discussed in tumour board meeting and planned for re-resection of involved deep margin. After re-resection, she has remained disease-free for last 3 years without any evidence of local or distant recurrence. In this case report, the importance of surgical resection with adequate margins, is highlighted and long term follow-up by physical examination and ultrasound for any local recurrence of DFSP in breast.
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http://dx.doi.org/10.29271/jcpsp.2018.08.645 | DOI Listing |
Int J Oral Maxillofac Surg
January 2025
Department of Oral and Maxillofacial Surgery, Tsukuba Gakuen Hospital, Tsukuba, Ibaraki, Japan.
Dermatofibrosarcoma protuberans (DFSP) is a low-grade, malignant, spindle cell tumour with an infiltrative growth pattern and a high local recurrence rate. Cases of oral DFSP are rare. This report describes a case of DFSP occurring in the labial mucosa.
View Article and Find Full Text PDFDermatofibrosarcoma 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.
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