Dermatofibrosarcoma protuberans (DFSP)[2] is a rare locally aggressive malignant cutaneous soft-tissue sarcoma.

In the Surveillance, Epidemiology and End Results (SEER) tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma.

Dermatofibrosarcoma protuberans begins as a minor firm area of skin most commonly about to 1 to 5 cm in diameter.

Commonly located on the chest and shoulders, the following is the site distribution of DFPS as was observed in Surveillance, Epidemiology, and End Results (SEER) database between 2000 and 2010.

[5][8] The World Health Organization in 2020 classified the fibro sarcomatous DFSP (DFSP-FS) variant (also termed dermatofibrosarcoma protuberans, fibro sarcomatous) 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.

The fibroblast, the cell of origin of this tumor, expresses the fusion gene in the belief that it codes for collagen.

In order to ensure that enough tissue is removed to make an accurate diagnosis, the initial biopsy of a suspected DFSP is usually done with a core needle or a surgical incision.

Due to the rarity, initial presentation of flat plaque (skin hardening) and the slow-growing nature of DFSP, it may be months to years without a protuberance (bump).

Immunohistochemical stains for CD34, S-100 protein, factor XIIIa, and estrogen and progesterone receptors were performed on biopsy specimens.

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.

[25] Treatment is primarily surgical, with chemotherapy and radiation therapy used if clear resection margins are not acquired.

Mohs micrographic surgery (MMS) has a high cure rate and lowers the recurrence reduction of DFSP[27] if negative resection margins are achieved.

[28] 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.

Inadequate initial treatment results in larger, deeper recurrent lesions, but these can be managed by appropriate wide excision.

It is suggested that imatinib may be a treatment for challenging, locally advanced disease and in the rare metastatic cases.

[35] Metastases to regional lymph nodes are rarer and are most likely in patients who have had multiple local recurrences after inadequate surgical resection.

The most extensive research study to date was Perspectives of Patients With Dermatofibrosarcoma Protuberans on Diagnostic Delays, Surgical Outcomes, and Nonprotuberance.