PAEDIATRIC LOW-GRADE GLIOMA (PLGG): UNDER THE KNIFE FOR A BETTER QUALITY OF LIFE. EVALUATION & PROGNOSTICATION OF LONG-TERM PLGG SURVIVOR FUNCTIONAL AND QUALITY OF LIFE OUTCOMES

Abstract AIMS PLGG is a generally survivable Paediatric CNS tumour. Previous research demonstrates an advantageous prog- nostic relationship between receipt & degree of surgical resection with OS and PFS, but there has been no large- cohort evaluation of the prognostic influence of surgical resection on PLGG functional or QOL outcomes. AIMS •Large-cohort analysis of long-term PLGG survivor functional & QOL outcomes: clinical follow-up assess- ment and survivor-reported QOL & function. •Prognostic evaluation of association between patient, tumour, treatment factors & PLGG survivor func- tional/QOL outcomes. METHODS Demographic, tumour & treatment factors of GOSH PLGG patients diagnosed 1980-2021 were retrospectively collected. Factors were prognostically evaluated with 1) clinically-assessed functional outcomes at last follow-up (vision, hearing, endocrine, neurocognitive & physical function), 2) current long-term survivor-reported PedsQl QOL scores and 3) current long-term survivor-reported function(PESAT-PT) questionnaires using multivariate logistic regression. RESULTS Within the PLGG cohort(N=814), OS was 94.3%, PFS 76%, median number treatments 1 (0-21), and 42% had >1 measured functional deficit at last clinical review (median 14yr follow-up). Young diagnostic age (<1yr), optic pathway tumours, absence of surgical resection, & receipt chemo/radiotherapy were independently associated with adverse clinically-assessed functional outcomes. 122 long-term survivors completed prospective PedsQl/PESAT-PT questionnaires at median duration 21 years from diagnosis. 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). On multivariable analysis, favourable Total PedsQl scores were associated with receipt of surgical resection(OR 5.94,p0.02) & fewer treatment interventions(OR 2.8,p0.0005). Adverse scores were associated with NF1(OR- 11.82,p0.04), BRAF-V600E mutation(OR-18.55,p0.04),OPG(OR-12.1, p0.01),& seizures(OR-18.93,p<0.0001). Receipt of surgical resection was the only factor prognostically associated with all clinically-measured function & survivor-reported function & QOL. CONCLUSION Receipt of surgical resection is prognostically-associated with superior OS,PFS, functional and QOL outcomes in long-term PLGG survivors, and may assist in reducing the influence of other adverse prognostic factors by preventing seizures and need for chemo/radiotherapy.