THU216 The Influence Of Age At Diagnosis On Clinical Presentation And Prognosis Of Childhood-onset Craniopharyngioma

Disclosure: J. Beckhaus: None. C. Friedrich: None. S. Boekhoff: None. G. Calaminus: None. B. Bison: None. M. Eveslage: None. B. Timmermann: None. J. Flitsch: None. H.L. Muller: None. The association between age at diagnosis (AaD) and clinical presentation and prognosis of pediatric patients with cra...

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Detalles Bibliográficos
Autores principales: Beckhaus, Julia, Friedrich, Carsten, Boekhoff, Svenja, Calaminus, Gabriele, Bison, Brigitte, Eveslage, Maria, Timmermann, Beate, Flitsch, Joerg, Muller, Hermann Lothar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Pediatric Endocrinology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10555099/
http://dx.doi.org/10.1210/jendso/bvad114.1466
Descripción
Sumario:Disclosure: J. Beckhaus: None. C. Friedrich: None. S. Boekhoff: None. G. Calaminus: None. B. Bison: None. M. Eveslage: None. B. Timmermann: None. J. Flitsch: None. H.L. Muller: None. The association between age at diagnosis (AaD) and clinical presentation and prognosis of pediatric patients with craniopharyngioma (CP) is not clear. Seven hundred and nine patients diagnosed with adamantinomatous CP have been recruited between 1999 and 2021 in HIT-ENDO, KRANIOPHARYNGEOM 2000/2007, KRANIOPHARYNGEOM Registry 2019 and prospectively observed. In our cohort study, we analyzed clinical presentation and outcome in these CP patients with regard to different AaD: Infants and toddlers (under 2 years), early childhood (2-6 years), middle childhood (6-12 years), and early adolescence (12-18 years). Following a median follow-up of 8.4 years, functional capacity (FMH), overall and event-free survival (EFS), and quality of life (QoL) (PEDQOL) were evaluated. 45.4% of the patients presented with severe obesity (BMI > 3SDS) at last visit. Children with AaD <6y showed lower EFS but higher QoL than children with AaD >6y. Reduced functional capacity (FMH) percentiles were linked to higher BMI-SDS at last visit (rho=-0.125, 95% CI [-0.21; -0.04]). Posterior hypothalamic involvement and surgical lesion were independent risk factors for both, severe obesity at last visit (OR=2.94, 95% CI [1.73;5.08]) and events (HR=1.59, 95% CI [1.12;2.26]). In the PEDQOL body image domain, patients with posterior hypothalamic involvement and lesion report lower scores. Nearly half of all patients suffer from sequelae of severe obesity and the hypothalamic syndrome. A diagnosis of CP before the age of 6 years may enable patients to adjust to their limitations earlier, but it may also increase the risk of relapse. The cause of severe obesity and a lower QoL is not AaD but posterior hypothalamic involvement of CP. Presentation: Thursday, June 15, 2023

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