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

Abstract

Context: Fifty percent of pediatric low-grade gliomas affect the optic pathway, hypothalamus, and suprasellar areas (OP/HSGs), resulting in significant long-term neuroendocrinopathy. Objective: This study aimed to dissect tumor-from treatment-related risk factors for OP/HSGassociated neuroendocrinopathy. Design: This was a retrospective case notes analysis of 166 children with newly diagnosed OP/HSGs at our quaternary center between 1980 and 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 survival (EEFS) were 47.2 and 20.8%, respectively. EEFS declined up to 15 years postdiagnosis, with hypothalamic involvement (P < .008) in earlier endocrinopathy; the reverse being true of its density (radiotherapy P = .001; hypothalamic involvement P < .006). GH deficiency (GHD) was most common (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 (P = .01), but replacement did not increase progression. CPP was associated with future GnD (P