Endocrine Abstracts

Craniopharyngiomas are paradoxically considered ‘benign’ and hence curable by complete excision. Despite a high survival rate, however, this notion belies a high morbidity and propensity for premature mortality from neuroendocrine disease and treatment complications in those diagnosed in childhood, whose disease differs from that in adulthood. These result from a proximity to, and invasion of, vital ophthalmic, neurometabolic, neurocognitive and neuroendocrine pathways, and the subsequent impact on normal growth, learning and maturation processes in the developing child. Some of these are life threatening, and all are life changing. The devastating effects of hypothalamic injury are poorly understood, whilst endocrine replacement therapy and neurocognitive rehabilitation are not always timely or streamlined into treatment pathways. In 2005, the first UK consensus management guidelines recognised the surgical contribution to hypothalamic injury and advocated conservative debulking strategies and up front radiation to stabilise disease, avoiding further surgical hypothalamic harm or that due to recurrence. Poor cognitive and endocrine outcomes have traditionally been blamed on cranial radiation rather than pre-existing disease (eg.diagnostic delays, recurrence) or treatment (eg.radical excision) variables, with increasing attempts to avoid or refine this in children. Since 2009, the theoretical cognitive advantages afforded by the reduced penumbral scatter of proton beam irradiation has made this the NHS UK standard of care, patients being sent to USA for therapy, without a risk-benefit analysis as compared with photons.We will present neuroendocrine and cognitive outcome data on our cohort of 54 patients treated on a conservative surgical and early radiation strategy over two decades, and compare these with our own historical, radical surgery series (published 1996) by treatment era, and with other published series. Our early longitudinal evidence suggests tumour position, size, recurrence and Paris grade of hypothalamic involvement at diagnosis, as well as the surgical and radiation strategy (early vs late) are greater influencers of outcome than type of radiation (protons vs photons), and a 2-staged surgical and early radiation strategy which stabilises disease, can reduce the neuroendocrine morbidity burden. Infants and children under 3 years at diagnosis are particularly vulnerable to poor outcomes, with greater prevalence of blindness at presentation, diagnostic delays, stroke, recurrent cystic tumours treated with experimental intracystic interferon, and avoidance of radiaition considered too neurotoxic. A considered 2-stage surgical strategy avoiding hypothalamic harm, timely radiation and intensive interval neuroendocrine / cognitive assessment and rehabilitation, are more likely to improve outcomes than radiation type.