QOL-15. PAEDIATRIC LOW-GRADE GLIOMA (PLGG): A LIFELONG DISEASE THAT BEGINS IN CHILDHOOD. EVALUATION AND PROGNOSTICATION OF LONG-TERM PLGG SURVIVOR FUNCTIONAL AND QUALITY OF LIFE OUTCOMES

Abstract

Abstract BACKGROUND For many patients, PLGG has a chronic disease course with potential for multiple and significant functional deficits and decreased Quality-of-Life (QOL). However, there have been limited large-cohort evaluations or prognostication studies of QOL & functional outcomes. AIMS 1. Large-cohort analysis of long-term PLGG survivor functional & QOL outcomes: combining clinical follow-up assessment with survivor-reported QOL & function. 2. Evaluation of prognostic associations between PLGG variables & functional/QOL outcomes. METHODS Demographic, tumour & treatment factors of PLGG patients diagnosed 1980-2021 at Great Ormond Street Hospital, UK, were retrospectively collected & evaluated prognostically with functional outcomes from last clinical follow-up.Cohort long-term survivors completed prospective QOL(PedsQl Core) & function(PESAT-PT) questionnaires.PedsQL scores were evaluated prognostically with patient/tumour/treatment factors using multivariate logistic regression.PedsQL scores were compared with healthy (N=649) & chronically-ill (N=137) reference populations. RESULTS PLGG cohort (N=814) OS was 94.3%,PFS 76% & median treatments 1 (0-21) at median follow-up 8 years. 42% had 1 functional deficit at last clinical follow-up. Diagnostic age, tumour location, surgical resection, & receipt chemo/radiotherapy were independently associated with functional outcomes. 122 long-term survivors completed prospective questionnaires at median follow-up 21 years. 87% reported dysfunction: physical 60%,visual 50%,neurocognitive 49%,social 25%, endocrine 23%,travel 21%,employment 18%,& hearing 11%. Mean PedsQl scores:Physical 82(0-100), Psychosocial 73(13-100), Total 76(9-100). PLGG survivors had significantly lower mean Total, Physical & Psychosocial PedsQl scores than healthy controls (p0.0001,p0.0003,p0.0001), but comparable to people with chronic illness (p0.60,p0.14,p0.21). No correlation identified between PedsQl scores & number of impaired functional domains. Favourable Total PedsQl scores were associated with surgical resection(OR-5.94,p0.02) & fewer treatment interventions on multivariate analysis(OR-2.8,p0.0005). Adverse PedsQl scores were associated with NF1 (OR-11.82,p0.04), BRAF-V600E mutation(OR-18.55,p0.04), OPGs(OR-12.1,p0.01), & seizures(OR-18.93,p<0.0001). CONCLUSIONS Prognostication of heterogeneous PLGG survivor functional and QOL outcomes is complex but possible, without clear correlation between QOL and functional outcomes. For many, PLGG is a lifelong disease that begins in childhood.