CNS embryonal tumour with PLAG family gene alteration: failure of response to RET inhibition
A rare central nervous system (CNS) embryonal tumour characterised by alterations in the PLAG family gene has recently been reported. RET is a direct PLAGL1/2 target and RET inhibitors were thought to have therapeutic potential in this tumour. In a patient with a CNS tumour with PLAGL2 amplification...
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| Autores principales: | , , , , , , |
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| Formato: | Article (Journal) |
| Lenguaje: | inglés |
| Publicado: |
April 2026
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| In: |
Neuropathology & applied neurobiology
Year: 2026, Volumen: 52, Número: 2, Pages: 1 |
| ISSN: | 1365-2990 |
| DOI: | 10.1111/nan.70076 |
| Acceso en línea: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1111/nan.70076 Verlag, lizenzpflichtig, Volltext: https://onlinelibrary.wiley.com/doi/abs/10.1111/nan.70076 
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| Notas de Autor: | David King, Annika Schneider, Bernhard Küster, David T.W. Jones, Daniel J.A. Connolly, Stephen Wharton, Michaela-Kristina Keck |
| Sumario: | A rare central nervous system (CNS) embryonal tumour characterised by alterations in the PLAG family gene has recently been reported. RET is a direct PLAGL1/2 target and RET inhibitors were thought to have therapeutic potential in this tumour. In a patient with a CNS tumour with PLAGL2 amplification, we show RET inhibition had no clinical effect. Transcriptomic profiling demonstrated low RET levels with associated low levels of RET phosphorylation (pRET). Measurement of RET/pRET activity may be useful in predicting response to RET inhibition in these tumours. |
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| Notas: | Gesehen am 26.08.2026 |
| Descripción Física: | Online Resource |
| ISSN: | 1365-2990 |
| DOI: | 10.1111/nan.70076 |