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Multifocal Dermatofibrosarcoma Protuberans

Journal: International Journal of Clinical & Experimental Otolaryngology (IJCEO) (Vol.2, No. 1)

Publication Date:

Authors : ; ; ; ;

Page : 19-22

Keywords : Dermatofibrosarcoma Protuberans; Multifocal; Surgery; Radiation; Non Immunocompromised;

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Abstract

Dermatofibrosarcoma protuberans (DFSP) is a superficial sarcoma stemming from fibroblastic and myofibroblastic differentiation within the dermis and underlying soft tissue. It is a very rare tumor with less than 4.5 cases per million persons per year in the US. Between 16 and 20% of the cases of DFSP may be associated with previous traumatic events ranging from mild ulcerations to bayonet stabbing. Most cases of DFSP (47%) occur in the trunk region, however roughly 10% of cases can be found in the head and neck region and 8% in the shoulder region. Metastasis is a rare occurrence, with a rate of roughly 2% over ten years, and usually occurs in the lung, brain, or bone. DFSP has the propensity to recur locally especially within the head and neck region where wide local excision is difficult to obtain without significant morbidity. Given the high risk of local recurrence adjuvant therapy in the form of radiation has grown in practice

Last modified: 2017-09-09 14:17:31