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Pediatric dermatofibrosarcoma protuberans: multi-institutional outcomes.

Corey W Iqbal¹, Shawn St Peter, Michael B Ishitani

¹Department of Surgery, Children's Mercy Hospitals and Clinics, Kansas City, Missouri, USA.

The Journal of Surgical Research

|March 25, 2011

Summary

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Dermatofibrosarcoma protuberans (DFSP) is a rare pediatric cancer. Prompt diagnosis and complete surgical excision with clear margins are crucial for successful treatment and preventing recurrence in children.

Area of Science:

Pediatric Oncology
Dermatopathology
Surgical Oncology

Background:

Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignancy in the pediatric population.
DFSP presents with variable clinical manifestations, potentially leading to diagnostic delays.

Purpose of the Study:

To review the clinical characteristics, treatment, and outcomes of pediatric DFSP.
To emphasize the importance of accurate diagnosis and complete surgical resection for managing pediatric DFSP.

Main Methods:

Retrospective review of patients aged 18 years or younger diagnosed with DFSP between 1980 and 2010.
Analysis of treatment modalities, including excisional biopsy and re-excision, and patient follow-up data.

Main Results:

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Fifteen pediatric patients were identified, with a female predominance.
The median time to diagnosis was 182 days; most patients underwent re-excision with negative margins.
No recurrences were observed in patients with clear margins after re-excision; no adjuvant therapy was administered, and there were no disease-related deaths.

Conclusions:

Pediatric DFSP is a rare malignancy requiring prompt diagnosis.
Complete surgical excision with clear margins is the cornerstone of curative treatment for pediatric DFSP.
Variable presentation can delay diagnosis, highlighting the need for vigilance in pediatric dermatology and oncology.