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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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Autori principali: King, David (Autore) , Schneider, Annika (Autore) , Küster, Bernhard (Autore) , Jones, David T. W. (Autore) , Connolly, Daniel J. A. (Autore) , Wharton, Stephen (Autore) , Keck, Michaela-Kristina (Autore)
Natura: Article (Journal)
Lingua:inglese
Pubblicazione: April 2026
In: Neuropathology & applied neurobiology
Year: 2026, Volume: 52, Fascicolo: 2, Pages: 1
ISSN:1365-2990
DOI:10.1111/nan.70076
Accesso online:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1111/nan.70076
Verlag, lizenzpflichtig, Volltext: https://onlinelibrary.wiley.com/doi/abs/10.1111/nan.70076
Testo
Note sull'autore:David King, Annika Schneider, Bernhard Küster, David T.W. Jones, Daniel J.A. Connolly, Stephen Wharton, Michaela-Kristina Keck
Descrizione
Riassunto: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.
Descrizione del documento:Gesehen am 26.08.2026
Descrizione fisica:Online Resource
ISSN:1365-2990
DOI:10.1111/nan.70076

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