778 A Systematic Review of Surgery for Optic Pathway Glioma

Abstract

Abstract Aim Optic pathway gliomas (OPG) are a rare group of primary CNS malignancies usually found in children. The contemporary role of surgery in managing OPG is unclear. This review aimed to systematically assess the contemporary role for surgery in managing OPGs with respect to survival and functional outcomes. Method A systematic review was conducted in accordance with the PRISMA guidelines. A broad search strategy was employed in the literature search of the major medical databases. Articles describing OPG patients (stratified by modified Dodge classification (mDC) location) undergoing tumour-directed surgery were included, whilst articles with <10 patients, published before 2000 and describing individual case reports, opinions or secondary research were excluded. Primary outcomes were overall survival (OS), progression-free survival (PFS), visual outcome and endocrine outcome. Results 1221 articles were originally identified, with 21 included in the qualitative synthesis. There were 13 Level II and 8 Level IV articles. 5-year OS and PFS was 94.6% and 57.8% respectively (n = 2139). Total resection of mDC I OPG was curative, with no other surgical strategy being associated with survival benefit. Surgery was not significantly associated with visual or endocrine outcome, aside from preservation of contralateral vision (p < 0.001) in one series and predicting posterior pituitary dysfunction and GH deficiency (both p = 0.05) in one cohort. Conclusions Patients undergoing surgery for OPG have excellent overall survival, but a significant proportion have disease progression and associated endocrinopathy. Although total resection of mDC I OPG appears curative, more robust evidence is required to ascertain the surgical role in OPG.