Dermatofibrosarcoma protuberans (DFSP)[2] is a rare locally aggressive malignant cutaneous soft-tissue sarcoma. DFSP develops in the connective tissue cells in the middle layer of the skin (dermis).[3] Estimates of the overall occurrence of DFSP in the United States are 0.8 to 4.5 cases per million persons per year.[4][5] In the United States, DFSP accounts for between 1 and 6 percent of all soft-tissue sarcomas[6] and 18 percent of all cutaneous soft-tissue sarcomas. In the Surveillance, Epidemiology and End Results (SEER) tumor registry from 1992 through 2004, DFSP was second only to Kaposi sarcoma.
^Kreicher, Kathryn L.; Kurlander, David E.; Gittleman, Haley R.; Barnholtz-Sloan, Jill S.; Bordeaux, Jeremy S. (January 2016). "Incidence and Survival of Primary Dermatofibrosarcoma Protuberans in the United States". Dermatologic Surgery. 42 (Suppl 1): S24–31. doi:10.1097/DSS.0000000000000300. ISSN1524-4725. PMID26730971. S2CID12966671.
^Kransdorf, M. J. (January 1995). "Malignant soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex, and location". American Journal of Roentgenology. 164 (1): 129–134. doi:10.2214/ajr.164.1.7998525. ISSN0361-803X. PMID7998525.