Dermatofibrosarcoma protuberans (DFSP) is a rare, soft-tissue neoplasm of low- to intermediate malignancy. It constitutes less than 0.1% of all malignant neoplasms and less than 2% of all soft-tissue sarcomas.^1,2 Although DFSP rarely metastasizes, it is highly invasive and locally aggressive.³

Epidemiology

DFSP is rare, with an estimated annual incidence of ~0.8 cases per million persons.^1,3 It typically affects young to middle-aged adults (20-50 years old), and it occurs with almost equal frequency in men and women.¹

Investigations of Targeted Therapy With Imatinib for Dermatofibrosarcoma Protuberans and

Treatment Options for DFSP: Imatinib

References :

1. Gloster HM, Jr. Dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996;35:355-374; quiz 375-376.

2. Mendenhall WM, Zlotecki RA, Scarborough MT. Dermatofibrosarcoma protuberans. Cancer. 2004;101:2503-2508.

3. National Comprehensive Cancer Network. Clinical Practice Guidelines in Oncology. Dermatofibrosarcoma. Version 2. 2005. Available at: http://www.nccn.org/professionals/physician_gls/PDF/dfsp.pdf. Accessed April 23, 2006.