UCL Discovery
UCL home » Library Services » Electronic resources » UCL Discovery

Neuroendocrine morbidity after pediatric optic gliomas: a longitudinal analysis of 166 children over 30 years

Gan, H-W; Phipps, K; Aquilina, K; Gaze, MN; Hayward, R; Spoudeas, HA; (2015) Neuroendocrine morbidity after pediatric optic gliomas: a longitudinal analysis of 166 children over 30 years. The Journal of Clinical Endocrinology & Metabolism , 100 (10) pp. 3787-3799. 10.1210/jc.2015-2028. Green open access

[thumbnail of Gan_ article template_UCL Discovery template 4.pdf]
Preview
Text
Gan_ article template_UCL Discovery template 4.pdf

Download (864kB) | Preview

Abstract

Context: 50% of pediatric low-grade gliomas affect the optic pathway, hypothalamus and suprasellar areas (OP/HSGs) resulting in significant long-term neuroendocrinopathy. Objective: To dissect tumor- from treatment-related risk factors for OP/HSG-associated neuroendocrinopathy. Design: Retrospective case notes analysis of 166 children with newly-diagnosed OP/HSGs at our quaternary center between 1980 –2010 by multivariate Cox, linear and logistic regression. Results: Patients were of median (range) age 4.9 (0.2–15.4) years at diagnosis and followed up for 8.3 (0.04 –26.8) years. Despite high 20-year overall survival (81.0%), progression-free and endocrine event-free (EEFS) survival were 47.2% and 20.8% respectively. EEFS declined up to 15 years postdiagnosis, with hypothalamic involvement (p0.001) being implicated more than radiotherapy (p0.008) in earlier endocrinopathy; the reverse being true of its density (radiotherapy p0.001; hypothalamic involvement p0.006). GH deficiency (GHD) was commonest (40.3%), followed by central precocious puberty (CPP, 26.0%), gonadotropin (GnD, 20.4%), TSH (13.3%), and ACTH (13.3%) deficiencies. GHD increased with later treatment eras (p0.01), but replacement did not increase progression. CPP was associated with future GnD (p0.05). Posterior pituitary dysfunction (PPD, 7.2%) occurred in 57.9% after only biopsies or shunt procedures, and was associated with 6/13 deaths. 50.2% became obese. Tumor extent, surgery and increased endocrinopathy, rather than radiotherapy, predicted visuo-cognitive morbidity. Conclusions: This first longitudinal OP/HSG-specific study demonstrates that hypothalamo-pituitary dysfunction evolves hierarchically over decades. Tumor location predicts its speed of onset and radiotherapy its density. GnD can evolve from previous CPP, whilst life-threatening PPD can occur after any surgery. Our data suggest that recent radiation-avoiding chemotherapeutic strategies have increased GHD without improving survival.

Type: Article
Title: Neuroendocrine morbidity after pediatric optic gliomas: a longitudinal analysis of 166 children over 30 years
Open access status: An open access version is available from UCL Discovery
DOI: 10.1210/jc.2015-2028
Publisher version: http://dx.doi.org/10.1210/jc.2015-2028
Language: English
Additional information: Copyright © 2015 by the Endocrine Society. All rights reserved.
Keywords: Pediatric optic glioma, juvenile pilocytic astrocytoma, survivorship, endocrine morbidity, neurology
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
URI: https://discovery.ucl.ac.uk/id/eprint/1471668
Downloads since deposit
108Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item