Article

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 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 (DFSP) is a rare, slow-growing fibrohistiocytic neoplasm that commonly favors young to middle-aged adults. It is most commonly seen on the trunk and frequently recurs locally after an incomplete excision, but distant metastasis is rare. Mohs micrographic surgery (MMS) is the treatment of choice for DFSP.
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    ABSTRACT: Congenital dermatofibrosarcoma protuberans (DFSP) is a rare tumor with varying clinical presentations that is commonly misdiagnosed. Treatment of congenital DFSP is complicated by delays in diagnosis and its propensity for subclinical spread. Of 61 reported cases, 11 (18%) were treated with Mohs micrographic surgery (MMS) and 46 (75%) were treated with wide local excision (WLE). One case was treated with imatinib, and the remaining 3 did not differentiate between receiving MMS or WLE. In the cases of congenital DFSP treated with MMS the clearance rate was 100% with an average follow-up of 4.3 years. The clearance rate seen with WLE was 89% with an average follow-up period of 1.9 years. The average margins taken during MMS (1.7 cm) were smaller than those taken with WLE (2.8 cm). Fifty percent of cases with available follow-up undergoing WLE required multiple surgeries. Based on superior cure rates with long-term follow-up, smaller surgical margins, and fewer surgical sessions, MMS should be considered as first-line treatment for congenital DFSP.
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