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Clinical and pathological features of pediatric dermatofibrosarcoma protuberans
T H Keshen1, P S Cederna, V H Savell
1Department of Surgery, University of Iowa College of Medicine, Iowa City 52242, USA.
Annals of Plastic Surgery
|December 1, 1995
Summary
Pediatric dermatofibrosarcoma protuberans (DFSP) is rare but treatable. Wide local excision or Mohs surgery offers excellent outcomes with no recurrences in a small study group.
Area of Science:
- Pediatric Oncology
- Dermatopathology
- Surgical Oncology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare malignant mesenchymal tumor known for local invasion and recurrence.
- DFSP in the pediatric population is exceptionally uncommon, with fewer than 50 reported cases.
Purpose of the Study:
- To define the clinical and pathological characteristics of DFSP in children.
- To review treatment options and outcomes for pediatric DFSP.
Main Methods:
- Retrospective review of seven pediatric patients diagnosed with DFSP.
- Analysis of clinical presentation, diagnostic procedures (biopsy types), pathological findings (histology, CD34 staining), and surgical treatments (wide local excision, Mohs micrographic resection).
Main Results:
- Patients presented with firm, slow-growing nodules fixed to the skin.
- Histology with CD34 staining confirmed diagnosis; most patients had positive margins after initial biopsy.
- No local recurrences or distant metastases were observed after a mean follow-up of 15.1 months following definitive surgery.
Conclusions:
- Pediatric DFSP exhibits specific clinical and pathological features.
- Aggressive surgical management, including wide local excision or Mohs surgery, appears effective in preventing recurrence and metastasis in children.