ISSN: 1859 - 2872
Challenges in the diagnosis and treatment of dermatofibrosarcoma protuberans: A case report
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Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous malignancy belonging to the group of soft tissue sarcomas. The pathogenesis of DFSP is associated with a chromosomal translocation t(17;22) that leads to fusion of the collagen type I alpha 1 (COL1A1) gene and platelet-derived growth factor beta (PDGFB) gene, resulting in tumor formation within the dermis. The disease progresses slowly over many years, with atypical clinical manifestations and significant local invasion, leading to a high recurrence rate after treatment. However, DFSP generally carries a favorable prognosis with a low rate of metastasis. Definitive diagnosis relies mainly on histopathological and immunohistochemical examinations. Current treatment guidelines recommend complete surgical excision as the first-line therapy. We report a case of a 38-year-old female diagnosed with localized DFSP of the right arm. The patient was treated with wide local excision and remained recurrence-free during a two-year follow-up period.
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References
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