Uwe Wollina1. 1. Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Dresden, Germany.
Abstract
BACKGROUND: 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. MAIN OBSERVATION: 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. CONCLUSIONS: 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.
BACKGROUND: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. MAIN OBSERVATION: 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. CONCLUSIONS: 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.
Authors: Victoria E Price; Jonathan A Fletcher; Maria Zielenska; William Cole; Sandra Viero; David E Manson; Mary Stuart; Alberto S Pappo Journal: Pediatr Blood Cancer Date: 2005-05 Impact factor: 3.167
Authors: A Paradisi; D Abeni; A Rusciani; E Cigna; M Wolter; N Scuderi; L Rusciani; R Kaufmann; M Podda Journal: Cancer Treat Rev Date: 2008-08-05 Impact factor: 12.111
Authors: Stephen N Snow; Ellen M Gordon; Paul O Larson; Mohammed M Bagheri; Michael L Bentz; David B Sable Journal: Cancer Date: 2004-07-01 Impact factor: 6.860