Dermatofibrosarcoma protuberans (DFSP) is a rare slow-growing cutaneous soft tissue sarcoma that is characterized to be locally invasive with a high recurrence rate. However, it has a poor metastatic capacity. This literature review was conducted to gather evidence-based medicine knowledge regarding DFSP. The literature search was conducted by using the keywords "Dermatofibrosarcoma Protuberans" and "soft tissue sarcomas" in the PubMed and Web of Science databases. Thirteen articles met the requirements and are included in the literature review. This review highlights the need for a high index of suspicion to help reach an early and proper diagnosis and to raise awareness. DFSP needs a specialized center in order to identify and recognize the disease. The knowledge and awareness of the disease is low since it is rare.
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