LAUSR

OBJECTIVE Craniopharyngiomas (CP) are rare malformational tumors. Clinical presentation and outcome of pediatric patients with CP with specific regard to age at diagnosis is not clear. The aim of this cohort study was to determine clinical presentation and outcome in these patients diagnosed at different ages at diagnosis. DESIGN Seven hundred and nine patients diagnosed with CP were recruited 1999-2021 in HIT-Endo, KRANIOPHARYNGEOM 2000/2007/Registry 2019 and prospectively observed. METHODS Age at diagnosis was categorized as infants&toddlers (<2y), early childhood (2-<6y), middle childhood (6-<12y) and early adolescence (12-<18y). Overall and event-free survival (EFS), functional capacity (FMH) and quality of life (QoL) (PEDQOL) were assessed. RESULTS Severe obesity (BMI >3SDS) was prevalent in 45.4% at last visit. Lower EFS but better QoL was observed in children with age at diagnosis <6y compared to ≥ 6y. Reduced functional capacity percentiles were associated with increased BMI-SDS at last visit (rho=-0.125, 95% CI [-0.21; -0.04]) and age at diagnosis <2y. Posterior hypothalamic involvement (HI) and hypothalamic lesion (HL) are independent risk factors for reduced event-free survival (HR=1.59, 95% CI [1.12; 2.26]) and obesity at last visit (OR=2.94, 95% CI [1.73; 5.08]). Age at diagnosis did not contribute to severe obesity and reduced QoL. CONCLUSIONS Diagnosis of CP at an age <6 years, may help patients to adapt early to disabilities, but may lead to a higher probability of CP relapse. Not age at diagnosis but posterior HL may be the contributing factor to severe obesity and a reduced QoL.