Detection of COL1A1-PDGFB fusion transcripts and PDGF α-and β-Receptors in giant cell fibroblastoma of the postsacrococcygeal region
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The definitive version is available at www.blackwell-synergy.comWe describe a case of recurrent giant cell fibroblastoma (GCF) of thepostsacrococcygeal region in a 2-year-old girl. Both the initial and recurrent tumorscontained solid and angiectoid areas. The former was composed of loosely arrangedwavy spindle cells and giant cells with a well vascularized myxoid to collagenousstroma. The angiectoid spaces were often lined by multinucleated giant cells.Immunohistochemically, the tumor cells and small vessels in the tumor tissue werepositive for platelet derived growth factor (PDGF)α- andβ-receptors. Molecularanalysis revealed fusion of collagen type Iα1 exon 26 with PDGF-B chain exon 2 thatinduced unscheduled production of PDGF-BB. These findings suggest that PDGF andits receptors significantly contribute to the development of GCF in an autocrine and aparacrine manner.
- Blackwell Publishingの論文
- 2006-05-00
Blackwell Publishing | 論文
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