Dermatofibrosarcoma protuberans (DFSP) is a fibroblast-derived intermediate-grade soft tissue sarcoma. It is usually observed in young to middle-aged adults, although it can occur in children as either a congenital or acquired lesion. DFSP is almost twice as common in individuals of African descent and has a slight female predominance. Up to 90% of DFSPs harbor a translocation t(17;22) resulting in a fusion of collagen 1a1 (COLL1A1) and platelet-derived growth factor B (PDGFB) genes, leading to constitutively activated tyrosine kinase signaling.

DFSP typically presents as a slow-growing, red-brown, indurated plaque with irregular nodularity in an adult. The most common site is the trunk, followed by the proximal extremities, head, and neck. In children, DFSP can appear atrophic, hypopigmented, or blue-red in color.

DFSP is an uncommon neoplasm with low metastatic potential, carrying a 2%-5% risk of distant metastasis. However, it can be locally aggressive and has a high rate of recurrence after surgical excision. Poor prognostic factors include older age, male sex, and larger tumor size. Approximately 9%-20% of tumors can undergo fibrosarcomatous degeneration.

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