Dermatofibrosarcoma protuberans in children

Department of Plastic & Reconstructive Surgery, The Children's Hospital at Westmead, Sydney, Australia.
Journal of Plastic Reconstructive & Aesthetic Surgery (Impact Factor: 1.47). 01/2008; 62(6):819-23. DOI: 10.1016/j.bjps.2007.11.009
Source: PubMed

ABSTRACT Dermatofibrosarcoma protuberans (DFSP) is a relatively rare neoplasm affecting the skin. It is an infiltrative tumour of intermediate malignancy, with a limited potential for metastasis but a high rate of recurrence. The incidence in children is even less frequent, although a proportion of those identified in adulthood may reflect a delay in diagnosis of childhood DFSP. We report the experience of DFSP seen at The Children's Hospital at Westmead (Sydney, Australia). Three children aged 5, 10 and 11 years of age underwent surgical excision of their lesions. Recurrence was evident in one child whose initial histopathology was not definitive for DFSP, and whose initial surgery had not involved wide local excision. All three children were male, and all had lesions affecting their trunk. One child whose lesion was thought to have been evident since birth may have represented congenital DFSP.

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    ABSTRACT: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant tumor. It is characterized by aggressive local infiltration, leading to a propensity for recurrence. In children, DFSP is even less common and likely misdiagnosed or underdiagnosed. This study is a review of DFSP in the pediatric population and aims to identify factors for successful treatment.
    Journal of Plastic Reconstructive & Aesthetic Surgery 06/2014; DOI:10.1016/j.bjps.2014.05.031 · 1.47 Impact Factor
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    ABSTRACT: Dermatofibrosarcoma protuberans is an uncommon, locally aggressive, cutaneous, soft tissue sarcoma. It is most comĀ­ monly seen on the trunk and it frequently recurs locally after an incomplete excision, but distant metastasis is rare. Patients need postĀ­operative local irradiation even with histopathological clear margins, following wide excision, owing to its high affinity for local recurrence. Here, we are reporting a case of recurrent dermatofibrosarcoma protuberans which was seen over the lower part of the abdomen.
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    ABSTRACT: Dermatofibrosarcoma protuberans is a rare mesenchymal malignancy in childhood and adolescence. The tumor is characterized by dermal spindle cell proliferation with infiltration of subcutaneous tissue, expression of CD34, and a specific fusion of the platelet-derived growth factor beta with the collagen type 1alpha1 gene. We observed a 10-year-old girl with a medaillon-like, asymptomatic plaque on the chest that was diagnosed as DSFP. The tumor was completely removed by delayed Mohs surgery. Follow-up so far has shown a complete response. The prognosis of dermatofibrosarcoma protuberans in children is excellent as long as early diagnosis is followed by complete excision with Mohs surgery as a golden standard.
    Journal of Dermatological Case Reports 12/2013; 7(4):121-4. DOI:10.3315/jdcr.2013.1160