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.
Trunk / Torso – 42%
Lower extremity – 21%
Upper extremity – 21%
Head and neck – 13%
Genitals – 1%

Variants
The World Health Organization, 2020, classified the fibrosarcomatous DFSP (DFSP-FS) variant (also termed dermatofibrosarcoma protuberans, fibrosarcomatous) of the dermatofibrosarcoma protuberans as a specific form of the intermediate (rarely metastasizing) fibroblastic and myofibroblastic tumors and other variants of this disorder as a specific form of the intermediate (locally aggressive) fibroblastic and myofibroblastic tumors. Bednar tumors
Bednar, or pigmented DFSP, is distinguished by the dispersal of melanin-rich dendritic cells of the skin. It represents 1-5 percent of all DFSP occurring in people with rich in melanin pigments. Bednar characterized by a dermal spindle cell proliferation like DFSP but distinguished by the additional presence of melanocytic dendritic cells. It occurs at the same rate of DFSP on fairer skin and should be considered to have the same chances of metastasis. Myxoid DFSP
Myxoid DFSP, which has areas of myxoid degeneration in the stroma

Giant cell fibroblastoma
Giant cell fibroblastoma that contains giant cells, and is also known as juvenile DFSP. Giant cell fibroblastomas are skin and soft tissue tumors that usually arise in childhood. They are sometimes seen in association with dermatofibrosarcoma protuberans (DFSP, hybrid lesions) or may transform or recur as DFSP. Atrophic DFSP
Atrophic DFSP resemble other benign lesions such as morphea, idiopathic atrophoderma, atrophic scar, anetoderma or lipoatrophy. It behaves like classic DFSP. It commonly favours young to middle aged adults. It has a slow infiltrative growth and a high rate of local recurrence if not completely excised.