An oral presentation of dermatofibromasarcoma protuberans with literature review: A case report

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally invasive soft tissue tumor with a high rate of recurrence after surgical intervention. DFSP primarily occurs on the trunk and proximal extremities, with reported 10-15% occurring in the head and neck region. DFSP presenting within the oral cavity is a rare entity with few descriptions in the literature. Standard of care involves resection, either by Mohs micrographic surgery or wide local excision with careful evaluation of the surgical margins. Due to the invasive nature of this tumor, complete excision in sensitive areas such as the head and neck pose unique challenges.

We present a case of DFSP presenting from within the oral cavity, along with its management, in a 50-year-old male. In addition, we review the published literature. The patient neglected a 3-month history of a rapidly enlarging mass of the left cheek. A buccal mucosal biopsy revealed the diagnosis of DFSP. The patient underwent wide local excision of the tumor resulting in a full thickness defect which required reconstruction utilizing a radial forearm free flap. The final pathology report demonstrated positive margins that necessitated additional excision.

There are few reported cases of an intraoral presentation of dermatofibrosarcoma. Due to the sensitive location of the tumor, there are challenges associated with obtaining clear margins after resection and ultimately reconstruction.

This case and literature review highlight the rarity of an oral cavity presentation DFSP, and the associated surgical challenges.