Background
Dermatofibrosarcoma protuberans (DFSP) in children is uncommon.
Methods
Retrospective review of patients ≤ 18 y with histopathologic diagnosis of DFSP from
1980–2010 treated at one of two tertiary referral centers.
Results
Fifteen patients were treated for pathologically-confirmed DFSP (mean ± SE age 11.9 ±
1.3 y). There was a female preponderance: 12 females, 3 males. Follow-up was available
in all patients over a median 5.8 y (range 0.2–20.1 y). Patients presented with subcutaneous
nodule (n = 4), cutaneous plaque (n = 3), or red papule (n = 3); the description of the lesion was not available for five patients. Abdominal
wall, lower extremity, and scalp were the most frequent sites involved (n = 4 each). Median time from onset of the lesion to pathologic diagnosis was 182 d
(range 5–1, 114 d). All 15 patients underwent initial excisional biopsy, and the diagnosis
of DFSP was made in 14. Thirteen patients then underwent re-excision (within a mean
± SE 20.4 ± 4.1 d) with negative margins. None of these patients had a recurrence.
There were two recurrences: one with positive margins on excisional biopsy who did
not have immediate re-excision; the other with a misdiagnosis on excisional biopsy.
Both underwent re-excision with negative margins and have not had any further recurrence.
No patients received adjuvant therapy. There were no disease-related deaths.
Conclusions
DFSP is a rare pediatric malignancy. Its presentation is variable, delaying diagnosis,
which is made with excisional biopsy. Excision with clear margins is critical for
cure.
Key Words
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Article info
Publication history
Published online: February 23, 2011
Received:
December 15,
2010
Identification
Copyright
© 2011 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
