Title

Pediatric dermatofibrosarcoma protuberans: multi-institutional outcomes.

Document Type

Article

Publication Date

9-1-2011

Identifier

DOI: 10.1016/j.jss.2011.01.042

Abstract

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.

Journal Title

The Journal of surgical research

Volume

170

Issue

1

First Page

69

Last Page

72

MeSH Keywords

Adolescent; Child; Child, Preschool; Dermatofibrosarcoma; Female; Humans; Male; Retrospective Studies; Skin Neoplasms

Keywords

DFSP; Children; Skin Cancer

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