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.
As with many other stromal neoplasms, DFSPs appear to express low levels of hormone receptors, which may be one factor that accounts for their accelerated growth during pregnancy. Treatment
Treatment is primarily surgical, with chemotherapy and radiation therapy used if clear resection margins are not acquired. Surgical treatment
The type of surgical treatment chosen is dependent on the location of the DFSP occurrence and possible size. Mohs surgery
Mohs Micrographic Surgery (MMS) has a high cure rate and lowers the recurrence reduction of DFSP if negative resection margins are achieved. Wide local excision
WLE was the gold standard for treating DFSP but is currently under reevaluation. Presently in the United States, WLE may be suggested after the recurrence of MMS. Larger resection margins are suggested for WLE than MMS. Recurrence rate with WLE is about 8.5% with a lower recurrence rate related to wider excision. Resection margin
DFSP characteristic features are its capacity to invade surrounding tissues, to a considerable distance from the central focus of the tumor in a "tentacle-like" fashion. This fact, coupled with diagnostic delay, may lead to inadequate initial resection. Inadequate initial treatment results in larger, deeper recurrent lesions, but these can be managed by appropriate wide excision. Radiation therapy
DFSP is a radioresponsive tumor, radiation therapy (RT) is not used as the first choice for treatment. Conservative resection through MMS or WLE is attempted first. If clear margins are not achieved RT, or Chemotherapy is recommended. Chemotherapy
DFSP was previously regarded and nonresponsive to standard chemotherapy.