Endocrine Abstracts

Introduction: Craniopharyngiomas are rare, pituitary tumours which, though benign with good survival, carry high neuroendocrine morbidity. Optimum management remains controversial despite a UK consensus strategy (2005).

Aim: To study disease- and treatment-related neuroendocrine, visual and cognitive outcomes in our most recent cohort, managed with individualised multidisciplinary decision making to limit hypothalamic morbidity.

Patients and methods: Retrospective longitudinal case review of 19 new patients to GOSH (12M and 7F) between 01/01/2009 and 31/12/2013, median age 7.43 (1.91–15.46) years. Patients’ endocrine, visual and cognitive outcomes were categorized by initial treatment strategy at diagnosis and last follow-up.

Results: At a median follow-up of 2.3 (0.7–4.7) years, there was an evolving endocrinopathy from diagnosis which was hierarchical (GHd 100% >TSHd 73.7% >ACTH 63.2% >DI 47.7% >high BMI-SDS (>+2) 10.5%) and aggravated by any surgical debulking rather than cyst aspiration, with little additional impact of radiation to residual disease. Our prevalence rates of ACTHd, DI and obesity are significantly lower than our own and other historic series (70–100%) at a shorter follow-up, without an increase in disease relapse rates (36.8%). All children maintained or improved their vision; only 6.2% were visually impaired. 78.9% received adjuvant radiation (nine protons, six IMRT), up front (12) or at recurrence (3), which effectively stabilised disease in 83.3%. Despite this in all seven patients with longitudinal cognitive assessments, performed at a median interval of 21.5 months, IQ remained unchanged.

Conclusion: Endocrine morbidity is caused by disease and surgery more often than radiation, which is highly effective in stabilising disease. Despite concerns of radiation neurotoxicity, especially in the youngest children, cognitive outcomes are currently stable and the rates of life-threatening DI, ACTHd and morbid obesity are significantly improved. Although there is a possibility that these may increase over time, rates are still lower than similar follow-up time from centres opting for a predominant surgical strategy (avoiding early radiation).