Pigmented Dermatofibrosarcoma Protuberans: Description of a pediatric case

Area of Science:

• Oncology
• Dermatopathology
• Pediatric Oncology

Background:

• Bednar tumor, a rare pigmented variant of dermatofibrosarcoma protuberans (DFSP), is a low-grade sarcoma.
• DFSP typically presents as a papulonodular lesion with slow, progressive growth.

Observation:

• A 9-year-old female presented with a 2-year history of an asymptomatic papule on her toe.
• Biopsy revealed pigmented DFSP, confirmed by CD34 positivity and abnormal PDGFB gene pattern via FISH.
• The patient underwent delayed Mohs surgery and skin grafting.

Findings:

• Pigmented DFSP is exceptionally rare in pediatric patients.
• Immunohistochemistry (CD34+) and molecular cytogenetics (FISH for PDGFB gene) aid diagnosis.
• Complete excision achieved no recurrence at 5-year follow-up.

Implications:

• Early suspicion and diagnosis of pigmented DFSP are crucial, especially in pediatric cases.
• Complete surgical excision is key to achieving a favorable prognosis.
• This case highlights successful management of a rare pediatric sarcoma.