Association for Academic Surgery| Volume 170, ISSUE 1, P69-72, September 2011

Pediatric Dermatofibrosarcoma Protuberans: Multi-Institutional Outcomes1

Published:February 23, 2011DOI:


      Dermatofibrosarcoma protuberans (DFSP) in children is uncommon.


      Retrospective review of patients ≤ 18 y with histopathologic diagnosis of DFSP from 1980–2010 treated at one of two tertiary referral centers.


      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.


      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

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of Surgical Research
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Chang C.K.
        • Jacobs I.A.
        • Salti G.I.
        Outcomes of surgery for dermatofibrosarcoma protuberans.
        Eur J Surg Oncol. 2004; 30: 341
        • Lemm D.
        • Mugge L.O.
        • Mentzel T.
        • et al.
        Current treatment options in dermatofibrosarcoma protuberans.
        J Cancer Res Clin Oncol. 2009; 135: 653
        • Kransdorf M.J.
        Malignant soft-tissue tumors in a large referral population: Distribution of diagnoses by age, sex, and location.
        Am J Roentgenol. 1995; 164: 129
        • Ratner D.
        • Thomas C.O.
        • Johnson T.M.
        • et al.
        Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans. Results of a multiinstitutional series with an analysis of the extent of microscopic spread.
        J Am Acad Dermatol. 1997; 37: 600
        • Miller S.J.
        • Alam M.
        • Andersen J.
        • et al.
        Dermatofibrosarcoma protuberans.
        J Natl Compr Cancer Network. 2007; 5: 550
        • Gloster Jr., H.M.
        • Harris K.R.
        • Roegnigk R.K.
        A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans.
        J Am Acad Dermatol. 1996; 35: 82
        • Weinstein J.M.
        • Drolet B.A.
        • Esterly N.B.
        Congenital dermatofibrosarcoma protuberans.
        Arch Dermatol. 2003; 139: 207
        • Farma J.M.
        • Ammori J.B.
        • Zager J.S.
        • et al.
        Dermatofibrosarcoma protuberans: How wide should we resect?.
        Ann Surg Oncol. 2010; (Mar 31 [epub ahead of print])
        • Allen A.E.
        • Tsou H.C.
        • Harrington A.
        • et al.
        Clonal origin of dermatofibrosarcoma protuberans.
        J Invest Dermatol. 1993; 100: 99
        • Bridge J.A.
        • Neff J.R.
        • Sanberg A.A.
        Cytogenetic analysis of dermatofibrosarcoma protuberans.
        Cancer Genet Cytogenet. 1990; 49: 199
        • Labropoulos S.
        • Razis E.D.
        Imantinib in the treatment of dermatofibrosarcoma protuberans.
        Biologics. 2007; 1: 347
        • Bendix-Hansen K.
        • Myhre-Jensen D.
        • Haae S.
        Dermatofibrosarcoma protuberans: A clinicopathological study of 19 cases and a review of world literature.
        Scand J Plast Reconstruct Surg Hand Surg. 1983; 17: 247
        • Burkhardt B.R.
        • Soule E.H.
        • Winkelmann R.K.
        • et al.
        Dermatofibrosarcoma protuberans: Study of fifty-six cases.
        Am J Surg. 1966; 111: 638
        • Pack G.T.
        • Tabah E.J.
        Dermatofibrosarcoma protuberans: A report of 39 cases.
        Arch Surg. 1951; 62: 391
        • Chaung T.Y.
        • Su W.P.D.
        • Muller S.A.
        Incidence of T cell lymphoma and other rare skin cancers in a defined population.
        J Am Acad Dermatol. 1990; 23: 254
        • Taylor H.B.
        • Helwig E.B.
        Dermatofibrosarcoma protuberans: A study of 115 cases.
        Cancer. 1962; 15: 717
        • McKee P.H.
        • Fletcher C.D.
        Dermatofibrosarcoma protuberans in infancy and childhood.
        J Cutan Pathol. 1991; 18: 241
        • Ahmed A.A.
        • Ostlie D.
        • Fraser J.D.
        • et al.
        Dermatofibrosarcoma protuberans in the breast of a 2-year-old girl.
        Ann Diagn Pathol. 2010; 14: 279
        • Ugurel S.
        • Kortmann R.D.
        • Mohr P.
        • et al.
        Short German guidelines: Dermatofibrosarcoma protuberans.
        J Dtsch Dermatol Ges. 2008; 6: S17