Cp-15. Long-Term Outcomes of Paediatric Craniopharyngiomas: Multi-Centre Comparative Study

Abstract BACKGROUND Craniopharyngioma (CP) account for 80% of childhood hypothalamopituitary tumours and, though benign, their proximity to the visual pathways, hypothalamus, and pituitary incurs significant long-term neurologic and endocrine morbidity. Informed surgical decision-making has the potential to reduce morbidity and improve outcomes. METHODS This multicentre retrospective observational study aimed to compare long-term outcomes of CP at two paediatric neurosurgery units (GOSH, UK, and USP, Brazil), delineating management paradigms associated with better prognosis. CP treated over a 20-year period were identified and standardized clinical outcomes recorded for comparative analysis. RESULTS 123 patients were identified (USP=52; GOSH=71). No demographic differences were identified between series (mean age: USP=9.2±4.6; GOSH=8.2±4.1 years). Headaches (USP=75%; GOSH=62%) and visual deficits (USP=46%; GOSH=38%) were the most common presenting symptoms. Growth hormone deficiency at presentation was more common in the USP cohort (USP=62.5%; GOSH=35%; p=0.009). There was significantly more hypothalamic invasion in the GOSH cohort (Paris grade 2: GOSH=67.0%; USP=37.5%; p=0.026), but significantly larger cystic volumes in the USP cohort (USP=27.5±37cm3; GOSH=10.5±8.9cm3;p=0.004). These differences led to different surgical approaches, with more subtotal resections at USP (78%) and more cyst aspirations at GOSH (62%). 80% of patients at GOSH received RT compared to 32% at USP. Mean follow-up at GOSH was 7.5±4.8 years compared to 10.1±5.6 years at USP. Cognitive outcome did not vary. GOSH cohort presented higher rates of levothyroxine replacement (USP=67%; GOSH=86%;p=0.017). GOSH exhibited better hypothalamic outcomes (p=0.08) and better visual outcomes (field deficits: GOSH=44%; USP=66%;p=0.01). In multivariate analysis (n=123), subtotal/gross-total resection (OR0.17; 95%CI:0.05-0.55;p0.01) and older age at diagnosis (OR0.91, 95%CI:0.82-0.99;p=0.04) were associated with longer progression-free survival. CONCLUSIONS Comparative analysis of two large craniopharyngioma series identified different paradigms of surgical management driven by clinical-radiological presentation with similar long-term outcomes. Subtotal/gross-total resection was not related to worse clinical outcome or better progression-free survival.