Dermatofibrosarcoma Protuberans: A Systematic Review of the Literature

Background: Dermatofibrosarcoma Protuberans (DFSP) is a rare slow growing cutaneous soft tissue sarcoma, that is characterized to be locally invasive with high recurrence rate. However, it has poor metastatic capacity. Objective: We are conducting this literature review to gather evidence-based medicine knowledge regarding Dermatofibrosarcoma Protuberans. Moreover, this study highlights the gaps of knowledge in regard to this disease entity. Methods: The literature search was conducted by searching the keywords “Dermatofibrosarcoma Protuberans” and “soft tissue sarcomas” in PubMed and Web of Science databases. Results: 1,769 potentially relevant results showed matched titles, of which 13 articles met the requirements and were included in the literature review. Limitations: Vague nonspecific clinical manifestations, makes it challenging to establish an early diagnosis and seek an appropriate counseling.  In addition, it is difficult to diagnose due to the slow growing behavior and the tumor’s benign appearance. As a result of all of that there is no enough cases reported in the literature. Conclusion: This review highlights the need of high index of suspicion to help reach an early and proper diagnosis and to raise awareness. Dermatofibrosarcoma Protuberans 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.