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Dermatofibrosarcoma protuberans in children.

Chaitan Reddy¹, Peter Hayward, Phillip Thompson

¹Department of Plastic & Reconstructive Surgery, The Children's Hospital at Westmead, Sydney, Australia. chaithan@gmp.usyd.edu.au

Journal of Plastic, Reconstructive & Aesthetic Surgery : JPRAS

|December 22, 2007

Summary

This summary is machine-generated.

Dermatofibrosarcoma protuberans (DFSP) is a rare pediatric skin cancer. Early diagnosis and wide surgical excision are crucial to prevent recurrence in children with this intermediate-grade malignancy.

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Area of Science:

Dermatopathology
Pediatric Oncology
Surgical Oncology

Background:

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally aggressive skin neoplasm.
DFSP has a high recurrence rate and low metastatic potential.
Pediatric DFSP is exceptionally rare, often leading to diagnostic delays.

Observation:

This study reviews three pediatric cases of DFSP treated at The Children's Hospital at Westmead.
All patients were male, presenting with trunk lesions at ages 5, 10, and 11.
One case suggested possible congenital DFSP, with a lesion present since birth.

Findings:

Recurrence occurred in one child due to non-definitive initial histopathology and inadequate surgical margins.
Surgical excision was performed for all three pediatric DFSP lesions.
The findings highlight the importance of accurate histopathological diagnosis and complete tumor removal.

Implications:

Emphasizes the need for vigilant diagnosis and appropriate management of pediatric DFSP.
Highlights the critical role of wide local excision in preventing recurrence.
Suggests considering congenital DFSP in infants with suspected early-onset skin tumors.