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[Myxoid dermatofibrosarcoma protuberans in childhood].
M Campos1, C Zarco, F Acquadro
1Servicio de Anatomía Patológica, Hospital Universitario 12 de Octubre, Madrid, Spain.
Actas Dermo-Sifiliograficas
|August 30, 2011
Summary
This study reports the first pediatric case of myxoid dermatofibrosarcoma protuberans on a finger. This rare skin tumor variant is typically seen in adults, highlighting its unusual presentation in a child.
Area of Science:
- Dermatopathology
- Pediatric Oncology
- Surgical Pathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive skin cancer.
- The myxoid variant of DFSP is uncommon and typically affects adults on the extremities.
- DFSP is characterized by spindle-shaped cells positive for CD34.
Observation:
- A case of myxoid dermatofibrosarcoma protuberans is presented in a 14-year-old girl.
- The tumor was located on the finger of the left hand and had been present for over 10 years.
- This represents the first reported pediatric case of myxoid DFSP at this anatomical site.
Findings:
- Microscopic examination revealed a neoplastic growth in the dermis and hypodermis with a prominent myxoid component.
- Infiltrating spindle cells at the periphery were diffusely CD34 positive.
- The differential diagnosis includes other CD34-positive myxoid mesenchymal tumors.
Implications:
- This case expands the known spectrum of DFSP presentation in pediatric patients.
- It underscores the importance of considering rare variants in the differential diagnosis of pediatric extremity tumors.
- Further research may clarify the behavior and management of pediatric myxoid DFSP.
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