A challenging fibrosarcomatous variant of dermatofibrosarcoma protuberans of head and neck: a case report

Abstract

Dermatofibrosarcoma protuberans (DFSP) is an unusual tumour of soft tissue with local invasive property with high rate of recurrence after surgical treatment. DFSP is frequently seen on the trunk and proximal extremities, although a 10-15% cases accounts in head and neck region. Mainstay for success of surgery in DFSP remains complete removal of tumour with adequate surgical margins of approximately 3cms. In head neck region challenges associated with surgery may be inadequate surgical margins, poor functional and cosmetic outcomes. We present a case of a 35 years old male reported to our hospital with complaint of rapidly growing mass over right cheek and retroauricular region causing facial deformity. Incisional biopsy diagnosis was made DFSP. The patient underwent wide local excision of tumour followed by adjuvant radiotherapy. Final histopatholgy showed fibrosarcomatous transformation of DFSP. After 24 months of post-op follow up, patient recovery was satisfactory without any signs of recurrence. This case is presented for its rarity and it highlights the need for proper diagnosis and treatment plan.