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Dfsp Research Paper

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Dermatofibrosarcoma protuberans (DFSP) was first described by Darier and Ferrand in 1924 [14]. DFSPs are rare monoclonal cutaneous soft tissue sarcomas of intermediate malignancy [1]. These locally aggressive spindle cell tumours tend to recur after conservative initial surgical intervention [2]. 10–15% of DFSP are high-grade variants associated with local recurrence and metastasis, and the remaining 85–90% are low-grade. It is a tumour of the deep dermis and subcutaneous tissue and when found in the breast, rarely involves the mammary glands. [16,19]

Epidemiology:
DFSP accounts for less than 0.1% of all malignancies and approximately 1% of all soft tissue sarcomas [14]. The incidence has been reported between 0.8–5.0 cases per 1 million persons per year. DFSP affects both men and women equally and has been documented in all races. DFSP most commonly occurs in adults between the ages of 20 and 50. Cases ranging from birth up to 80 years of age have been reported, but these are exceedingly rare. [4,14]

Pathogenesis:
Trauma has been documented as a triggering factor in 10 to 20% of cases. Reports also describe tumour development in scars from previous operations, burn wounds, varicella or BGC immunizations, and rapid growth during pregnancy (due to progesterone receptors in the tumour). DFSP has been …show more content…

They are initially asymptomatic and slowly progressive but many go on to ulcerate, bleed or become painful. These lesions grow slowly over months to several decades and eventually develop rubbery nodules within plaques. Growth often accelerates once nodules appear [14]. These lesions usually vary in size from 1–5 cm but can reach 20 cm or more in advanced cases. [10,14] DFSP are most commonly located on the trunk (50 to 60%), proximal extremities (20%), and head and neck (10 to 15%). [13] Acral and genital DFSPs are

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