Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare neoplasmof the dermislayer of the skin, and is classified as a sarcoma. In many respects, the disease behaves as a benigntumor, but in 2-5% of cases it can metastasize, so it should be considered to have malignantpotential.

Over 95% of DFSP tumors have the chromosomal translocationt(17;22). The translocation fuses the collagengene(COL1A1) with the platelet-derived growth factorgene. The fibroblast, the cellof origin of this tumor, expresses the fusion genein the belief that it is collagen. However the resulting fusion proteinis processed into mature platelet-derived growth factorwhich is a potent growth factor. Fibroblasts contain the receptorfor this growth factor. Thus the cell "thinks" it is producing a structural protein, but in fact produces a self-stimulatory growth signal. The cell dividesrapidly and a tumor forms.

Treatment is primarily surgical, with chemotherapyand radiation therapysometimes being used.

There is clinical evidence that imatinib, which inhibits PDGFB, may be effective for tumors positive for the t(17;22) translocation.

External link

• Thesis on DFSP

This article is licensed under the GNU Free Documentation License.
It uses material from the http://en.wikipedia.org/wiki/Dermatofibrosarcoma+protuberans Wikipedia article Dermatofibrosarcoma protuberans.