Dermatology Online Journal

Dermatofibrosarcoma protuberans (DFSP), a rare medium grade sarcoma, occasionally occurs in childhood and is even more rarely present at birth. In children, the clinical appearance may be mistaken for a vascular malformation and so delayed diagnosis is not uncommon. Dermatofibrosarcoma protuberans is locally invasive and notorious for its high recurrence rate even after attempted wide local excision owing to extensive subclinical and asymmetrical extensions. In adult DFSP, Mohs Micrographic Surgery (MMS) is the treatment of choice because it offers a higher clearance rate compared to wide local excision. However, MMS may result in extended operating times owing to tissue processing and multiple stages. In children, this means a prolonged period under general anesthetic, which may be undesirable.  We describe an interesting case of a 4- year-old girl diagnosed with DFSP. She underwent a modified MMS procedure in which she had two short general anesthetics.  The advantage of MMS technique in which the full peripheral and deep margin of the specimen was examined.