ADULT HISTONE G34 MUTANT GLIOMAS - A SINGLE CENTRE EXPERIENCE OVER 10 YEARS

Abstract AIMS Histone G-34-mutant (H3 G34) gliomas are rare, aggressive infiltrating tumours of the cerebral hemispheres and classified as WHO grade 4 paediatric type diffuse high-grade glioma. They most commonly affect children adolescents. We describe clinicopathological characteristics adult H3 G34-mutant seen at National Hospital for Neurology Neurosurgery over last 10 years. METHOD retrospectively analyzed clinic radiological data on all cases identified from neuropathology database. Data were extracted patient demographics, clinical features outcome. RESULTS Six patients (2 male, female) identified, The median age onset was 26 years (range 17-38 years). One presented with a Motor Neurone Disease syndrome (progressive asymmetric spastic tetraparesis bulbar symptoms), two cognitive decline three symptoms raised intracranial pressure. All supratentorial - 2 had mass lesions typical glioma, 1 imaging consistent an intraventricular ependymoma. 3 findings that suggested non-tumour diagnosis extensive white matter disease more in keeping inflammatory demyelinating process or leucodystrophy. overall survival 23 Months 68 months). CONCLUSIONS Adult have variable presentation associated prognosis similar to other gliomas. should be considered differential presenting predominantly disorder. Multi-centre studies needed better delineate phenotype prognostic features.