Childhood dermatofibrosarcoma protuberans: role of preoperative imaging

Abstract

Dermatofibrosarcoma protuberans (DFSP) is especially challenging to recognize and diagnose in children. Prompt and definitive treatment is essential based on the tumor's high rate of recurrence. Our purpose is to increase physician awareness and recognition of DFSP in the pediatric population with the largest case series of childhood DFSP to our knowledge thus reported in the literature. We also propose that magnetic resonance imaging (MRI) may be useful in the preoperative assessment. The records of 10 patients with primary DFSP between the ages of 8 months and 16 years were reviewed. Only 1 of 10 patients was given the correct diagnosis at the initial medical evaluation. Of the 10 cases, 5 were congenital. The average delay in diagnosis was 5 years. Of the patients, 5 had preoperative MRI to delineate the size and extent of the tumor. In the two cases with the largest tumors preoperative MRI significantly altered surgical planning and execution. Thus, preoperative MRI may be helpful in planning for successful surgical clearance of large tumors and preventing local recurrence. All 10 cases were treated with Mohs micrographic surgery and no recurrences were noted with a follow-up range of 6 weeks to 17 years.