Image and content excerpted from the VisualDx clinical decision support system.
VisualDx images show variation in age, skin color, and disease stage. VisualDx has 36 images of Dermatofibrosarcoma Protuberans.
Full text and additional images for Dermatofibrosarcoma Protuberans are available in VisualDx.
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C44.90 – Unspecified malignant neoplasm of skin, unspecified
276799004 – Dermatofibrosarcoma protuberans
SynopsisDermatofibrosarcoma 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.