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Published on: May 6, 2016
Platelet-derived growth factor receptor expression and amplification in choroid plexus carcinomas
Nina N Nupponen1, Janna Paulsson, Astrid Jeibmann
1Molecular Cancer Biology Program, University of Helsinki, Helsinki, Finland.
Platelet-derived growth factor (PDGF) receptors are frequently expressed and amplified in pediatric choroid plexus carcinomas. This finding supports targeting PDGF receptor signaling for novel treatments in these rare, aggressive tumors.
Area of Science:
- Oncology
- Molecular Biology
- Pediatric Cancer Research
Background:
- Platelet-derived growth factor (PDGF) receptor signaling is linked to glial tumor development.
- Choroid plexus carcinomas (CPCs) are rare pediatric tumors with poor prognosis.
- Novel therapeutic targets for CPCs are urgently needed.
Purpose of the Study:
- To investigate the expression and gene amplification status of PDGF receptors alpha and beta in CPCs.
- To explore the potential of PDGF receptor signaling as a therapeutic target in CPCs.
Main Methods:
- Immunohistochemistry was used to assess PDGF receptor alpha and beta protein expression in 22 CPC patient samples.
- Chromogenic in situ hybridization (CISH) was employed to determine gene copy number status for PDGFRA and PDGFRB.
- Statistical analysis, including Spearman correlation, was performed to evaluate relationships between protein expression, gene amplification, and patient survival.
Main Results:
- High protein expression scores for PDGF receptor alpha were observed in 27% of CPCs, and for PDGF receptor beta in 59%.
- Copy-number gains of PDGFRA were found in 8/12 evaluable cases, with one case showing amplification.
- PDGFRB amplification was detected in 5/12 cases, and its amplification status significantly correlated with PDGF receptor beta protein expression (P=0.01).
- No significant association was found between PDGF receptor expression and progression-free survival.
Conclusions:
- PDGF receptor alpha and beta are frequently expressed and their genes amplified in choroid plexus carcinomas.
- PDGF receptor beta shows a significant correlation between gene amplification and protein expression.
- These findings provide a strong rationale for developing PDGF receptor signaling-targeted therapies for CPCs.
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