Sclerosing DFSP
Sclerosing DFSP is a variant in which the cellularity is low, and the tumor consists of uniform bundles of collagen interspersed with more typical DFSP cells.Granular cell variant is a rare type in which spindle cells are mingled with richly granular cells, the granules being lysosomal, with prominent nucleoli. Fibrosarcomatous DFSP (DFSP-FS)
Fibrosarcomatous DFSP, a rare variant of DFSP involving greater aggression, high rates of local occurrences, and higher metastatic potential. DFSP - FS are considered to be intermediate-grade sarcomas. Although they rarely metastasize (fewer than 5 percent of cases)

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 (PDGF) 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 a 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 creates a self-stimulatory growth signal. The cell divides rapidly and tumor forms. The tissue is often positive for CD34. Diagnosis
DFSP is a malignant tumor diagnosed with a biopsy, when a portion of the tumor is removed for examination.
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