Pediatric Dermatofibrosarcoma Protuberans: Multi-Institutional Outcomes1

BackgroundDermatofibrosarcoma protuberans (DFSP) in children is uncommon.MethodsRetrospective review of patients ≤ 18 y with histopathologic diagnosis of DFSP from 1980–2010 treated at one of two tertiary referral centers.ResultsFifteen 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.ConclusionsDFSP 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.