Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous neoplasm associated with a high cure rate. We present a case of aggressive DFSP with fibrosarcomatous areas in the head and neck. A 28-year-old Mediterranean female presented with a 45-day history of rapidly growing cutaneous lesion of the face. Surgical biopsy confirmed the diagnosis of DFSP. Subsequently, the patient underwent wide local surgical resection, followed by reconstruction. Histopathology report revealed fibrosarcomatous transformation and the patient underwent adjuvant radiotherapy. The patient continues to be disease free at the 35-month follow-up.Although DFSP behave as non-aggressive malignancy, surgery with complete removal of the affected area is the intervention of choice. Moreover, adjuvant treatment and follow-up of the patient is essential in order to prevent recurrence.
Highlights
Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive, cutaneous, malignant tumor characterized by high propensity for local relapse and low metastatic potential
It was first recognized by Taylor [1] in 1890, and described by Darrier [2] in 1924, but the term “dermatofibrosarcoma protuberans” was coined by Hoffman [3] in 1925
We report a rare case of an aggressive head and neck DFSP with fibrosarcomatous areas
Summary
Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive, cutaneous, malignant tumor characterized by high propensity for local relapse and low metastatic potential. It has been reported to involve many body surfaces, mainly the trunk (42-72%), followed by the extremities (16-30%) and less commonly in the head and neck (10-16%) [4] It constitutes less than 0.1% of all malignant neoplasms, it represents the most frequent skin sarcoma (nearly 1% of all soft tissue sarcomas), more than 1% of all head and neck malignant tumours and 7% of all head and neck sarcomas [5,6]. The decision was made, in a multi-disciplinary meeting, to treat the tumour with wide local resection This involved a superficial parotidectomy and selective neck dissection (levels II-V). Histopathological examination, of the completely excised tumour, revealed a nodular neoplastic lesion composed of large spindle-shaped cells with prominent nuclei, showing a high mitotic index and areas of necrosis, infiltrating into the subcutaneous fat and muscles (Figure 5). At 35-month post-treatment follow-up, the patient continues to be symptom free with no signs of tumour recurrence
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