Dermatofibrosarcoma protuberans: review of 20-years experience

RESUMEN

No disponible

ABSTRACT

Introduction. Dermatofibrosarcoma protuberans(DFSP) is an uncommon soft tissue neoplasm withlow-intermediate grade of malignancy. It is a locallyaggressive tumour with a high recurrence rate. Surgicalexcision with adequate margins is the maintreatment.Materials and methods. We describe the clinicopathologicalfeatures of 21 cases of DFSP. Themean size of the lesions was 5.6 cm, mostly locatedin trunk. 61.9% of the cases underwent sugical excisionwithout previous biopsy. 52.4% of the patientspresented positive margins, that required surgicalextension.Results. The recurrence rate was 28.6% (6 cases),five of them local recurrences treated with new surgicalexcision with wide margins. Median periodfree of illness was 32.5 months. Medium follow-upperiod was 33.25 months.Conclusions. DFSP has a locally high recurrencerate, that has been associated to inadequate surgicalmargins. The histological knowledge preoperativelywould permit surgical excision with adequatemargins, and probably this could reduce the recurrencerate. Radiotherapy could avoid the surgicalextension of margins in these cases with positiveones. Chemotherapy could be indicated in metastasiccases. Most recurrences appear in the first 3years, but it is important a long-term follow-up ofthese patients