Another Rare Case of Oral Dermatofibrosarcoma Protuberans with Review of the Literature

Elizabeth McDougal, Dr. Carlos Martinez, Dr. Jarom Gilstrap, Dr. Mariangela Rivera

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 originating from the oral mucosa is a rare entity with few descriptions in the literature. Standard of care involves resection, either by Mohs micrographic surgery or wide local excision. Due to the invasive nature of this tumor, wide margins in sensitive areas such as the head and neck pose unique challenges.  We present a rare case of DFSP involving the oral mucosa in a 50-year-old male who presented with a 3-month history of a rapidly enlarging mass of his left cheek. He subsequently underwent buccal mucosa biopsy, which demonstrated a spindle cell tumor that was later determined to be DFSP. He underwent a wide local excision resulting in a full thickness defect which required reconstruction utilizing a radial forearm free flap. However final pathology demonstrated positive margins requiring re-excision. This case highlights the rarity of mucosal DFSP, and the associated surgical challenges encountered with DFSP.