Dermatofibrosarcoma Protuberans

Image and content excerpted from the VisualDx clinical decision support system.


Image of Dermatofibrosarcoma Protuberans

VisualDx images show variation in age, skin color, and disease stage. VisualDx has 25 images of Dermatofibrosarcoma Protuberans.

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ICD Codes

ICD-9-CM:
173.90 – Unspecified malignant neoplasm of skin, site unspecified

ICD-10-CM:
C44.90 – Unspecified malignant neoplasm of skin, unspecified

Synopsis

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.

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.

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