Dermatofibrosarcoma Protuberans: An Unusual Parotid Swelling - A Case Report

Abstract

Background: Dermatofibrosarcoma protuberans (DFSP) is a slowly growing tumor. It has a high recurrence rate. The tumor is characterized by dermal spindle cell proliferation with infiltration of subcutaneous tissue, and expression of CD34 on immunnohistochemistry is pathgnomic. Main observation: We observed a 46-years-old female patient complaining of recurrent fixed swelling in the parotid region that was diagnosed as DSFP. The tumor had been surgically excised and the patient was referred for adjuvant radiotherapy and a follow-up every month for 2 years with no reported recurrence. Conclusion: Although rare, DFSP requires a high index of suspicion if there is recurrent painless, cutaneous, and multinodular lesion in the parotid region. In this context, wide local excision (>3 cm margins) combined with post-operative radiotherapy plays a fundamental role in the management of DFSP patients.