Premium
Detection of COL1A1 – PDGFB fusion transcripts and platelet‐derived growth factor α and β receptors in giant cell fibroblastoma of the postsacrococcygeal region
Author(s) -
Kashima A.,
Yamashita A.,
Moriguchi S.,
Marutsuka K.,
Tsumori S.,
Yoshizato K.,
Ishihara A.,
Setoyama M.,
Asada Y.
Publication year - 2006
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.2006.07146.x
Subject(s) - pdgfb , platelet derived growth factor receptor , platelet derived growth factor , giant cell , autocrine signalling , paracrine signalling , growth factor , biology , pathology , growth factor receptor , receptor , microbiology and biotechnology , cancer research , medicine , genetics
Summary We describe a 2‐year‐old girl with recurrent giant cell fibroblastoma (GCF) of the postsacrococcygeal region. Both the initial and recurrent tumours contained solid and angiectoid areas. The former was composed of loosely arranged wavy spindle cells and giant cells with a well‐vascularized myxoid to collagenous stroma. The angiectoid spaces were often lined by multinucleated giant cells. Immunohistochemically, the tumour cells and small vessels in the tumour tissue were positive for platelet‐derived growth factor (PDGF) α and β receptors. Molecular analysis revealed fusion of collagen type I α 1 exon 26 with PDGF‐B chain exon 2 that induced unscheduled production of PDGF‐BB. These findings suggest that PDGF and its receptors significantly contribute to the development of GCF in both an autocrine and a paracrine manner.