Pathophysiology
More than 90% of DFSP tumors have the chromosomal translocation t(17;22). The translocation fuses the collagen gene (COL1A1) with the platelet-derived growth factor gene. The fibroblast, the cell of origin of this tumor, expresses the fusion gene in the belief that it codes for collagen. However the resulting fusion protein is processed into mature platelet-derived growth factor which is a potent growth factor. Fibroblasts contain the receptor for this growth factor. Thus the cell "thinks" it is producing a structural protein, but it actually produces a self-stimulatory growth signal. The cell divides rapidly and a tumor forms.
The tissue is often positive for CD34.
Read more about this topic: Dermatofibrosarcoma Protuberans