Dermatofibrosarcoma protuberans (DFSP) is a rare coetaneous sarcoma that originates from fibroblasts of dermis and presents as a non tender coetaneous nodule. Local slow invasion, recurrence after therapy and low risk of metastasis are its main specifications. Pathology and immunohistochemistry are used to diagnose and wide surgery is the best approach for treatment. We present a case of recurrent DFSP and review the diagnostic and therapeutic modalities of this rare tumor. The patient was a 46 year old woman with 14 years history of a tumoral lesion in antero-superior part of her left shoulder and several recurrences after 4 excisional surgery for her lesion. Pathological and immunohistochemical surveys confirmed the diagnosis of DFSP. Previous studies performed on DFSP showed that it is a rare malignant coetaneous lesion that early diagnosis and appropriate treatment can lead to a better prognosis.

Keywords: Dermatofibrosarcoma protuberans, pathology, CD34

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