{"title":"10岁儿童隆突性皮肤纤维肉瘤。","authors":"Uwe Wollina","doi":"10.3315/jdcr.2013.1160","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Main observation: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":15601,"journal":{"name":"Journal of dermatological case reports","volume":"7 4","pages":"121-4"},"PeriodicalIF":0.0000,"publicationDate":"2013-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.3315/jdcr.2013.1160","citationCount":"3","resultStr":"{\"title\":\"Dermatofibrosarcoma protuberans in a 10-year-old child.\",\"authors\":\"Uwe Wollina\",\"doi\":\"10.3315/jdcr.2013.1160\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>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.</p><p><strong>Main observation: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>\",\"PeriodicalId\":15601,\"journal\":{\"name\":\"Journal of dermatological case reports\",\"volume\":\"7 4\",\"pages\":\"121-4\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2013-12-30\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://sci-hub-pdf.com/10.3315/jdcr.2013.1160\",\"citationCount\":\"3\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of dermatological case reports\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.3315/jdcr.2013.1160\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2013/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of dermatological case reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.3315/jdcr.2013.1160","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2013/1/1 0:00:00","PubModel":"eCollection","JCR":"","JCRName":"","Score":null,"Total":0}
Dermatofibrosarcoma protuberans in a 10-year-old child.
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.
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