CNS Embryonal Tumour With PLAG Family Gene Alteration: Failure of Response to RET Inhibition

Neuropathology and Applied Neurobiology 2026

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Authors: David King, Annika Schneider, Bernhard Küster, David T. W. Jones, Daniel Connolly, Stephen Wharton, Michaela‐Kristina Keck
DOI: 10.1111/nan.70076
Cited by: 3
References: 16

Abstract

Summary 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.

Fields of Study & Subjects

Pituitary Gland Disorders and Treatments Glioma Diagnosis and Treatment Genetic Syndromes and Imprinting

Publication details

Journal / source
Neuropathology and Applied Neurobiology
Publisher
Wiley
Volume
52
Issue
2
Pages
e70076–e70076
Publication date
1 April 2026
Language
EN