## 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 __COL1A1βPDGFB__. This same fusion gene is also seen in the supernumerary ring chromosome of dermatofibrosarcoma protuberans
Gains of COL1A1-PDGFB genomic copies occur in fibrosarcomatous transformation of dermatofibrosarcoma protuberans
β Scribed by Abbott, Jared J; Erickson-Johnson, Michele; Wang, Xiaoke; Nascimento, Antonio G; Oliveira, Andre M
- Book ID
- 109888514
- Publisher
- Nature Publishing Group
- Year
- 2006
- Tongue
- English
- Weight
- 220 KB
- Volume
- 19
- Category
- Article
- ISSN
- 0893-3952
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## Abstract Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous malignant tumor that usually occurs in the trunk, extremities, head, and neck but very rarely in the breast. Fibrosarcomatous transformation of DFSP is even rarer, with a higher risk of distant metastasis and poorer prognosis th
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