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Liegl, B; Leithner, A; Bauernhofer, T; Windhager, R; Guelly, C; Regauer, S; Beham, A.
Immunohistochemical and mutational analysis of PDGF and PDGFR in desmoid tumours: is there a role for tyrosine kinase inhibitors in c-kit-negative desmoid tumours?
Histopathology. 2006; 49(6):576-581 Doi: 10.1111/j.1365-2559.2006.02562.x
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Führende Autor*innen der Med Uni Graz
Liegl-Atzwanger Bernadette
Co-Autor*innen der Med Uni Graz
Bauernhofer Thomas
Beham Alfred
Gülly Christian
Leithner Andreas
Regauer Sigrid
Windhager Reinhard

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AIM: To determine the platelet-derived growth factor (PDGF) alpha and beta status of desmoid tumours. Desmoid tumours are rare monoclonal neoplasms that appear to have no metastatic potential. Surgical resection and radiotherapy in the event of a positive surgical margin is the first-line treatment. Recurrences are frequent. Treatment results using non-steroidal anti-inflammatory agents, anti-oestrogen compounds and other agents such as Imatinib mesylate have been published. Therapy with Imatinib has been proposed as a therapeutic option, although in most reports desmoid tumours are reported to be c-kit-. METHODS AND RESULTS: We performed immunohistochemical analysis on 124 archived samples (85 patients) of desmoid tumours using antibodies to PDGFalpha, PDGFbeta, PDGFRalpha and PDGFRbeta. All desmoid tumours showed immunoreactivity with antibodies to PDGFalpha and PDGFRalpha, whereas with antibodies to PDGFbeta and PDGFRbeta no specific reaction could be detected. Mutational analysis of PDGFRalpha (exons 11, 12, 17 and 18) and PDGFRbeta (exon 12) on frozen material from 14 patients was performed, but no mutations leading to amino acid changes in the mature protein were identified. CONCLUSION: The absence of an activating mutation in a protooncogene does not exclude the efficacy of tyrosine kinase inhibitors through other possible mechanisms, and these might be a therapeutic option for patients with desmoid tumours in whom established local and systemic approaches fail to control the disease.
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Adult -
DNA Mutational Analysis -
DNA, Neoplasm - analysis
Enzyme Inhibitors - metabolism
Female - metabolism
Fibromatosis, Abdominal - genetics
Humans - genetics
Immunohistochemistry - methods
Male - methods
Platelet-Derived Growth Factor - genetics
Protein-Tyrosine Kinases - antagonists and inhibitors
Proto-Oncogene Proteins c-kit - metabolism
Proto-Oncogene Proteins c-sis - genetics
Receptor, Platelet-Derived Growth Factor alpha - metabolism
Receptor, Platelet-Derived Growth Factor beta - metabolism
Receptors, Platelet-Derived Growth Factor - genetics
Tumor Markers, Biological - metabolism

Find related publications in this database (Keywords)
PDGFR alpha
PDGFR beta
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