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Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans

โœ Scribed by Barry M. Shmookler; Franz M. Enzinger; Sharon W. Weiss


Publisher
John Wiley and Sons
Year
1989
Tongue
English
Weight
1023 KB
Volume
64
Category
Article
ISSN
0008-543X

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## Abstract Giant cell fibroblastoma (GCF) is a subcutaneous mesenchymal neoplasm characterized by the chromosomal t(17;22), which results in the formation of the fusion gene __COL1A1โ€PDGFB__. This same fusion gene is also seen in the supernumerary ring chromosome of dermatofibrosarcoma protuberans

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Dermatofibrosarcoma protuberans (DFSP) and its juvenile form, giant-cell fibroblastoma (GCF), are uncommon infiltrative tumors of the dermis, which present unique cytogenetic features, such as the reciprocal translocation t(17;22) or, more commonly, supernumerary ring chromosomes containing sequence