Review
Dermatofibrosarcoma protuberans with fibrosarcomatous transformation of the head and neck
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* Corresponding author: Nikolaos Angouridakis nikos.angouridakis@gmail.com
1 Otorhinolaryngology, Head and Neck Surgery Department, AHEPA University Hospital, Thessaloniki, Greece
2 Department of Oral Surgery and Radiology, School of Dentistry, Aristotle University, Thessaloniki, 541 24, Greece
3 Department of Head and Neck Surgery, Barnet and Chase Farm Hospitals NHS Trust, London, UK
4 Department of Pathology, Aristotle University of Thessaloniki, Thessaloniki, Greece
5 Otorhinolaryngology, Head & Neck Surgery Department, Papanikolaou General Hospital of Thessaloniki, Thessaloniki, Greece
Head & Neck Oncology 2011, 3:5 doi:10.1186/1758-3284-3-5
Published: 4 February 2011Abstract
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.