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Childhood dermatofibrosarcoma protuberans: role of preoperative imaging
Sharon Longshore Thornton1, Janet Reid, Francis A Papay
1Division of Dermatologic Surgery, Department of Dermatology, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.
Insights
Dermatofibrosarcoma protuberans (DFSP) is difficult to diagnose in children, often leading to delayed treatment. This study highlights the importance of physician awareness and magnetic resonance imaging (MRI) for better pediatric DFSP management.
Area of Science:
- Pediatric Oncology
- Dermatology
- Radiology
Background:
- Dermatofibrosarcoma protuberans (DFSP) presents diagnostic challenges in pediatric patients.
- Prompt diagnosis and treatment are crucial due to DFSP's high recurrence rate.
Observation:
- This case series reviewed 10 pediatric patients with primary DFSP, ages 8 months to 16 years.
- Only one patient received an accurate initial diagnosis; the average diagnostic delay was 5 years.
- Five cases were congenital, and five underwent preoperative MRI to assess tumor extent.
Findings:
- Preoperative MRI significantly impacted surgical planning for large tumors, aiding in complete clearance.
- All 10 patients were treated with Mohs micrographic surgery.
- No recurrences were observed during follow-up periods ranging from 6 weeks to 17 years.
Implications:
- Increased physician awareness is needed for earlier recognition of pediatric DFSP.
- Magnetic resonance imaging (MRI) can be a valuable tool for preoperative assessment of pediatric DFSP.
- Mohs micrographic surgery appears effective in achieving recurrence-free outcomes for pediatric DFSP.
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.
