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Dermatofibrosarcoma Protuberans
Treatment
Surgery is the mainstay of treatment for DFSP. A wide excision with margins of 3 cm or more is recommended due to its irregular and frequently deep extensions. In one study, wide resection of primary DFSP was associated with a local recurrence-free rate of 100% at 54 months, whereas wide resection of patients who presented with recurrent DFSP lesions, which were larger and deeper and more likely to involve bone, demonstrated an 80% recurrence rate.1 Unfortunately, wide excision may result in functional impairment and cosmetic defects, with substantial adverse impact on quality of life.2
Recently, Mohs micrographic surgery has been increasingly accepted as the treatment of choice. Mohs micrographic surgery uses successive fixed-tissue sections to guide surgical removal.3 This procedure is relatively tissue sparing, but it is labor intensive and not readily applied to large tumors.2,4,5
Radiation has occasionally been used as a primary treatment for DFSP. Its primary utility, however, is in an adjuvant setting.6 Adjuvant radiotherapy may be of particular value post-surgically in patients with close or positive margins. Its value in patients with macroscopic disease is limited.2,7
Course
DFSP is highly invasive and locally aggressive. It has a significant risk of local recurrence, resulting in substantial morbidity associated with aggressive local invasiveness.
DFSP rarely metastasizes: 1% to regional sites and 4-5% to distant metastasis.5 When DFSP does metastasize, the prognosis is grim; the majority of patients die within 2 years.4,8
Emerging Therapies
Recent advances in the understanding of the molecular pathogenesis of DFSP have led to the development of a new therapeutic approach based on targeted inhibition of the PDGFRB tyrosine kinase. This approach shows considerable promise in the management of patients with DFSP. Clinical trials are underway.
Clinical Trials
For U.S. Residents Only
References:
- Khatri VP, Galante JM, Bold RJ, et al. Dermatofibrosarcoma protuberans: reappraisal of wide local excision and impact of inadequate initial treatment. Ann Surg Oncol. 2003;10:1118-1122.
- Stojadinovic A, Karpoff HM, Antonescu CR, et al. Dermatofibrosarcoma protuberans of the head and neck. Ann Surg Oncol. 2000;7:696-704.
- Mohs FE. Chemosurgery. Clin Plast Surg. 1980;7:349-360.
- McArthur G. Molecularly targeted treatment for dermatofibrosarcoma protuberans. Semin Oncol. 2004;31:30-36.
- Snow SN, Gordon EM, Larson PO, et al. Dermatofibrosarcoma protuberans: a report on 29 patients treated by Mohs micrographic surgery with long-term follow-up and review of the literature. Cancer. 2004;101:28-38.
- 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.
- Mendenhall WM, Zlotecki RA, Scarborough MT. Dermatofibrosarcoma protuberans. Cancer. 2004;101:2503-2508.
- Maki RG, Awan RA, Dixon RH, Jhanwar S, Antonescu CR. Differential sensitivity to imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans. Int J Cancer. 2002;100:623-626.
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