Abstract
Introduction
Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue neoplasm with low-intermediate grade of malignancy. It is a locally aggressive tumour with a high recurrence rate. Surgical excision with adequate margins is the main treatment.
Materials and methods
We describe the clinicopathological features of 21 cases of DFSP. The mean size of the lesions was 5.6 cm, mostly located in trunk. 61.9% of the cases underwent sugical excision without previous biopsy. 52.4% of the patients presented positive margins, that required surgical extension.
Results
The recurrence rate was 28.6% (6 cases), five of them local recurrences treated with new surgical excision with wide margins. Median period free of illness was 52.5 months. Medium follow-up period was 55.25 months.
Conclusions
DFSP has a locally high recurrence rate, that has been associated to inadequate surgical margins. The histological knowledge preoperatively would permit surgical excision with adequate margins, and probably this could reduce the recurrence rate. Radiotherapy could avoid the surgical extension of margins in these cases with positive ones. Chemotherapy could be indicated in metastasic cases. Most recurrences appear in the first 3 years, but it is important a long-term follow-up of these patients.
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References
Chang CK, Jacobs IA, Salti GI. Outcomes of surgery fordermatofibrosarcoma protuberans. J Clin Oncol. 1996;14:2565–9.
Gloster HM Jr.Dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996;55: 375–6.
Lindner NJ, Scarborough MT, Powell GJ, Spanier S, Enneking WF. Revision surgery indermatofibroma protuberans of the trunk and extremities. Eur J Surg Oncol. 1999;25:392–7.
Abenoza P, Lillemoe T. CD34 and factor XIIIa in the differential diagnosis of dermatofibroma anddermatofibrosarcoma protuberans. Am J Dermatopathol. 1993;15 (5):429–34.
Bowne WB, Antonescu CR, Leung DH, et al.Dermatofibrosarcoma protuberans. A clinicopa thologic analysis of patients treated and followed at a single institution. Cancer. 2000;88:2711–20.
Rutgers EJ, Kroon BB, Albus-Lutter CE, Gortzak E.Dermatofibrosarcoma protuberans: treatment and prognosis. Eur J Surg Oncol. 1992;18:241–8.
Enzinger FM, Weiss SW. Fibrohistiocytic tumors of intermediate malignancy. En: Stamathis G. Soft tissue tumors. St Louis: Mosby; 1988. p. 252–68.
Mendenhall WM, Zlotecki RA, Scarborough MT.Dermatofibrosarcoma protuberans. Cancer. 2004;101(11):2503–8.
Ballo MT, Zagars GK, Pisters P, Pollack A. The role of radiation therapy in the management ofdermatofibrosarcoma protuberans. Int J Radiat Oncol Biol Phys. 1998; 40:823–7.
Rubin BP, Schuetze SM, Eary JF, et al. Molecular targeting of Platelet-Derived Growth Factor B by Imatinib Mesylate in a patient withdermatofibrosarcoma protuberans. J Clin Oncol. 2002;20(17):3586–91.
Labropoulos SV, Fletcher JA, Oliveira AM, Papadopoulos S, Razis ED. Sustained emplete remission of metastaticdermatofibrosarcoma protuberans with imatinib mesylate. Anticancer Drugs. 2005;16(4): 461–6.
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Ruiz-Tovar, J., Guarino, M.F., Callejas, M.E.R. et al. Dermatofibrosarcoma protuberans: review of 20-years experience. Clin Transl Oncol 8, 606–610 (2006). https://doi.org/10.1007/s12094-006-0067-z
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DOI: https://doi.org/10.1007/s12094-006-0067-z