Outcome after pediatric craniopharyngioma: the role of age at diagnosis and hypothalamic damage
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Outcome after pediatric craniopharyngioma: the role of age at diagnosis and hypothalamic damage. / Beckhaus, Julia; Friedrich, Carsten; Boekhoff, Svenja; Calaminus, Gabriele; Bison, Brigitte; Eveslage, Maria; Timmermann, Beate; Flitsch, Jörg; Müller, Hermann L.
In: EUR J ENDOCRINOL, Vol. 188, No. 3, 02.03.2023, p. 300–309.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Outcome after pediatric craniopharyngioma: the role of age at diagnosis and hypothalamic damage
AU - Beckhaus, Julia
AU - Friedrich, Carsten
AU - Boekhoff, Svenja
AU - Calaminus, Gabriele
AU - Bison, Brigitte
AU - Eveslage, Maria
AU - Timmermann, Beate
AU - Flitsch, Jörg
AU - Müller, Hermann L
N1 - © The Author(s) 2023. Published by Oxford University Press on behalf of (ESE) European Society of Endocrinology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
PY - 2023/3/2
Y1 - 2023/3/2
N2 - 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 from 1999 to 2021 in HIT-Endo and KRANIOPHARYNGEOM 2000/2007/Registry 2019 and prospectively observed.METHODS: Age at diagnosis was categorized as infants and toddlers (<2 years), early childhood (2-6 years), middle childhood (6-12 years), and early adolescence (12-18 years). Overall and event-free survival (EFS), functional capacity (FMH), and quality of life (QoL) (PEDQOL) were assessed.RESULTS: Severe obesity (body mass index [BMI] >3 standard deviation score [SDS]) was prevalent in 45.4% at last visit. A lower EFS but better QoL was observed in children with age at diagnosis <6 years compared with ≥6 years. Reduced functional capacity percentiles were associated with increased BMI-SDS at last visit (rho = -0.125, 95% confidence interval [CI; -0.21; -0.04]) and age at diagnosis <2 years. Posterior hypothalamic involvement and hypothalamic lesion (HL) were independent risk factors for reduced EFS (hazard ratio = 1.59, 95% CI [1.12-2.26]) and obesity at last visit (odds ratio = 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 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.CLINICAL TRIAL REGISTRATION NUMBERS: NCT00258453; NCT01272622; NCT04158284.
AB - 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 from 1999 to 2021 in HIT-Endo and KRANIOPHARYNGEOM 2000/2007/Registry 2019 and prospectively observed.METHODS: Age at diagnosis was categorized as infants and toddlers (<2 years), early childhood (2-6 years), middle childhood (6-12 years), and early adolescence (12-18 years). Overall and event-free survival (EFS), functional capacity (FMH), and quality of life (QoL) (PEDQOL) were assessed.RESULTS: Severe obesity (body mass index [BMI] >3 standard deviation score [SDS]) was prevalent in 45.4% at last visit. A lower EFS but better QoL was observed in children with age at diagnosis <6 years compared with ≥6 years. Reduced functional capacity percentiles were associated with increased BMI-SDS at last visit (rho = -0.125, 95% confidence interval [CI; -0.21; -0.04]) and age at diagnosis <2 years. Posterior hypothalamic involvement and hypothalamic lesion (HL) were independent risk factors for reduced EFS (hazard ratio = 1.59, 95% CI [1.12-2.26]) and obesity at last visit (odds ratio = 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 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.CLINICAL TRIAL REGISTRATION NUMBERS: NCT00258453; NCT01272622; NCT04158284.
KW - Adolescent
KW - Child
KW - Child, Preschool
KW - Humans
KW - Infant
KW - Cohort Studies
KW - Craniopharyngioma/complications
KW - Neoplasm Recurrence, Local
KW - Obesity/complications
KW - Obesity, Morbid
KW - Pituitary Neoplasms/diagnosis
KW - Quality of Life
U2 - 10.1093/ejendo/lvad027
DO - 10.1093/ejendo/lvad027
M3 - SCORING: Journal article
C2 - 36857103
VL - 188
SP - 300
EP - 309
JO - EUR J ENDOCRINOL
JF - EUR J ENDOCRINOL
SN - 0804-4643
IS - 3
ER -
