Gan, HW;
(2013)
Patient-, disease- and treatment-related factors implicated in late neuroendocrine morbidity after paediatric optic pathway gliomas.
Masters thesis , UCL Institute of Child Health.
Abstract
Background. 30-50% of paediatric low-grade gliomas affect the optic pathway or suprasellar midline (OPGs), resulting in significant tumour- or treatment-related neuroendocrine dysfunction despite their benign histology. This study uniquely reports on the predictors of endocrinopathy in a large single-centre OPG cohort over 30 years of follow-up. Methods. Retrospective case note review of 166 OPG patients at Great Ormond Street Hospital between 1980 and 2010. Results. Patients were of median age 4.9 (0.2-15.4) years at diagnosis and followed-up for a median of 8.2 (0.04-29.7) years. 20-year overall, progression-free and endocrine event-free (EEFS) survival were 84.9%, 49.0% and 20.8% respectively, with EEFS falling up to 15 years from diagnosis, being independently reduced by hypothalamochiasmatic tumour involvement (p=0.008) more than radiotherapy (p=0.03). Conversely, the number of deficits was increased more by radiotherapy (p=0.007) than hypothalamic involvement (p=0.02), with progression over time. EEFS was lower in more recent treatment eras (p=0.01). GH deficiency was commonest (41.0%), followed by central precocious puberty (26.0%), gonadotrophin deficiency (20.4%), ACTH deficiency (14.5%), TSH deficiency (13.3%) and posterior pituitary dysfunction (PPD, 13.3%). PPD was increased by repeated surgeries (including shunt insertions or biopsies, p=0.03). 32.5% developed obesity, particularly those with hypothalamic tumours (p=0.02). Conclusions. This long-term multivariate analysis of OPG survivors provides new evidence suggesting that tumour location is more predictive of early endocrinopathy than irradiation, but radiotherapy increases its severity. It challenges the benefit of more recent treatment strategies, which do not improve survival but compound this risk, particularly as even minor surgery can result in life-threatening PPD.
| Type: | Thesis (Masters) |
|---|---|
| Title: | Patient-, disease- and treatment-related factors implicated in late neuroendocrine morbidity after paediatric optic pathway gliomas |
| Event: | UCL Institute of Child Health |
| Keywords: | Optic pathway glioma, juvenile pilocytic astrocytoma, low-grade, neuroendocrine late effects, survival |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices 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 > 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/1447831 |
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