ECE2023 Poster Presentations Endocrine-related Cancer (62 abstracts)
1Klinikum Oldenburg AöR, Department of Paediatrics and Paediatric Heamtology/Oncology, University Childrens Hospital, Oldenburg, Germany; 2University Hospital Bonn, Bonn, Germany; 3University Hospital Augsburg, Augsburg, Germany; 4University of Münster, Institute of Biostatistics and Clinical Research, Münster, Germany; 5Essen University Hospital, Department of Particle Therapy, Essen, Germany; 6University Medical Center Hamburg-Eppendorf, Department of Neurosurgery, Hamburg, Germany
Background: Adamantinomatous craniopharyngiomas (CP) are rare malformational tumors. The association between age at diagnosis and the outcome, clinical presentation and treatment of pediatric CP patients is not clear. The aim of this cohort study was to determine clinical presentation, outcome and quality of life in CP patients diagnosed at different AaD.
Methods: Seven hundred and twenty-two patients diagnosed with CP were recruited 1999-2021 in HIT-Endo, KRANIOPHARYNGEOM 2000/2007/Registry2019 and prospectively observed. AaD was categorized as infants and toddlers (<2y), early childhood (2<6y), middle childhood (6<12y) and early adolescence (12<18y). After median follow-up of 8.4 years, overall and event-free survival (EFS), functional capacity (FMH) and quality of life (QoL) (PEDQOL) were assessed. Multivariable cox regression was applied to compare the survival of three age groups in dependence of HI and HL. Multivariable logistic regression was used to determine potential risk factors for obesity (BMI SDS >3) at last visit.
Results: In this cohort, the prevalence of severe obesity (BMI >3SDS) was 45.4%. No differences regarding surgical approach were determined between the age groups. Lower EFS but better QoL was observed in children with AaD <6y compared to □6y. Reduced functional capacity (FMH) percentiles were associated with increased BMI-SDS at last visit (rho=-0.125, 95% CI [-0.21; -0.04]) and AaD <2y. Posterior HI and HL are independent risk factors for events (HR=1.59, 95% CI [1.12;2.26]) and obesity at last visit (OR=2.94, 95% CI [1.73;5.08]). Patients with posterior HI and HL report worse scores on PEDQOL body image domain.
Conclusions: Severe obesity is a frequent sequela in nearly half of all pediatric CP patients. Diagnosis of CP at an age <6 years, enables patients to adapt earlier to disabilities, but increases the probability of CP relapse. Posterior HI and HL can lead to obesity (BMI SDS >3), which reduces the functional capacity and self-evaluation on body image. Therefore, GTR should be viewed critically.