Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue sarcoma primarily found on the trunk and proximal extremities that typically appears as a slowly progressing, firm, violet-red, or blue plaque. Because DFSP is a slow-growing tumor, the diagnosis is often delayed for months to years. Most cases of DFSP are associated with a t(17;22) (q22;q13) translocation, generating the fusion protein COL1A1-PDGFB.
Extensive local infiltration, extending well beyond the visible lesion, characterizes DSFP, with distant metastasis being rare unless fibrosarcomatous transformation occurs. Surgical excision with clear margins is the preferred treatment, utilizing techniques like Mohs micrographic surgery or wide local excision.Radiation therapy can decrease local recurrence, particularly in cases where wide margins are impractical. More recently, tyrosine kinase inhibitors have shown promising response rates in patients with advanced or metastatic disease. Patients require vigilant monitoring for local recurrence following curative treatment.
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