Dermatofibrosarcoma Protuberans

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Image of Dermatofibrosarcoma Protuberans

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C44.90 – Unspecified malignant neoplasm of skin, unspecified

276799004 – Dermatofibrosarcoma protuberans


Dermatofibrosarcoma protuberans (DFSP) is a fibroblast-derived low-grade sarcoma. It presents as a slow-growing, asymptomatic skin-colored plaque with irregular nodularity on the trunk or proximal extremity of a middle-aged individual. It is more common in individuals of African descent and has a slight male predominance. May occur in tattoos.

Although it is an uncommon neoplasm with low metastatic potential, DFSP can be locally aggressive and has a high rate of recurrence after surgical excision.

A 2012 and 2013 study revealed a unique t(17;22)(q22;q13) translocation in 90% of DFSP tumors that fuses the collagen 1A1 (Col1A1) and platelet-derived growth factor B (PDGFB) genes, leading to constitutively activated tyrosine kinase signaling.

Pediatric Patient Considerations:
Giant cell fibroblastoma is a variant of DFSP that affects children.

For more information, see OMIM.

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