Dermatofibrosarcoma Protuberans on the Occipital Scalp Showed Uncommon Presentation: A Case Report

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma, with an incidence of about 0.8% to 5% per million people per year, accounting for 1% of soft tissue sarcomas. In its early stage, DFSP is typically found as a violet or pinkish macule or patch, and it can develop into a palpable mass with ulceration or bleeding. The standard treatment for DFSP is wide local excision of the tumor with a 2- to 3-cm negative margin, and radiation therapy or chemotherapy can be conducted with surgical treatment. A 35-year-old man had a palpable mass on the left side of his occipital scalp without color change, ulceration, or bleeding, which typically are present in malignancy. A magnetic resonance imaging (MRI) scan showed a 3-cm homogenous enhanced mass without adhesion between the scalp and the mass. Unexpectedly, a biopsy revealed the round mass to be DFSP. A wide excision and rotation of the scalp flap were performed. The patient recovered without any complications and received adjuvant radiotherapy at a dose of 60 Gray (Gy) for six weeks. There was no recurrence through six months of follow-up. Here we report this unique case of DFSP with atypical presentation.