Myxoaid dermatofibrosarcoma protuberans: a rare diagnosis and review of litrature

Pal Naresh., Griwan M S., Beniwal Meenu and Tayal Nikhil

Dermatofibrosarcoma protuberans is a very rare slow growing deceptive neoplasm. It is reported that the classic form of dermatofibrosarcoma has a very limited metastatic potential, while a fibrosarcomous transformation of dermatofibrosarcoma protuberans has a much higher potential. Dermatofibrosarcoma Protuberans (DFSP) represents a low grade, locally aggressive mesenchymal neoplasm with characteristic clinicopathologic, immunohistochemical, and molecular findings. Myxoid DFSP is an uncommon variant, with only few cases reported in the literature and may present a diagnostic challenge on histopathologic examination. It has potential to develop local recurrence as well as metastases especially to the lungs. A 27-year-old man presented with soft tissue swelling on right of temporal region for one month. It was 3X2 cm. of size and firm on consistency. The swelling was mobile on underlying structures and was painless. The provisional diagnosis of sebaceous cyst was made and excision biopsy done with full margins of overlying skin and deep up to muscle. We report here the case of myxoid DFSP along with a review of literature arising in the scalp describing the morphologic and immunohistochemical findings. Recognition of this variant at unusual sites is clinically important because the differential diagnosis includes benign and malignant tumors which could lead to under or over-treatment.

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Pal Naresh, et al,2017, Myxoaid dermatofibrosarcoma protuberans: a rare diagnosis and review of litrature, International Journal of Current Science and Technology, Vol.5, Issue, 5, pp. 427-430