Endocrine Abstracts

JOINT338

Background: Craniopharyngiomas are benign suprasellar tumours, approximately 4% of childhood brain tumours. Despite their WHO grade I classification, craniopharyngiomas have a high burden of morbidity due to their location and treatment-related sequelae, including Hypothalamic Syndrome (HS). HS is a rare but significant disorder, caused by tumour and/or treatment related injury to the hypothalamus, consisting of hypo/hyperphagia, hypothalamic obesity, pituitary dysfunction, sleep disturbance, behavioural changes and temperature disturbance, causing morbidity and reduced quality of life. This single specialised centre cohort study evaluates the burden of hypothalamic syndrome in paediatric patients with craniopharyngioma.

Method: A case notes review was undertaken for those diagnosed with craniopharyngioma <16 years old identified on the cancer registry at a single regional centre from 1/1/2014 to 1/1/2024. A data proforma was created after discussion with the multi-disciplinary team.

Results: 19 patients were identified, median age at diagnosis 8 years (IQR 5-10 years), males: 10/19, 52. 6%, females 9/19, 47. 4%. All patients underwent neurosurgery, 7 recieved proton radiotherapy and 5 recieved photon radiotherapy. Patients were followed up for a median time of 6 years 3 months (range 1. 5-10. 3 years). All patients had at least 2 hormone deficiencies (growth hormone and adrenocorticotropic hormone deficiencies). 15/19 had four or more hormone deficiencies. 57. 9% of patients with craniopharyngioma had ≥3 symptoms of hypothalamic dysfunction. All patients were asked about appetite and hunger, and 8 patients reported hyperphagia, and 2 patients reported hypophagia. Of those with hyperphagia, half (n = 4) were diagnosed with hypothalamic obesity. Thirteen patients (68. 4%) reported sleep issues mainly difficulty falling asleep and snoring. Temperature dysregulation was only documented as having been discussed in two patients, one who reported feeling cold. Behavioural issues were documented as having been discussed in 17 patients (89. 5%), with 8 patients having behavioural issues, manifesting as anxiety in three patients. Mortality in this patient group was 0% at the time of this service evaluation. There was a significant burden of HS in this group, with the median number of features of hypothalamic syndrome 3, ranging from 1-5 symptoms of hypothalamic syndrome out of a total possible six features of HS.

Conclusion: Patients with craniopharyngioma are at very high risk of hypothalamic syndrome, necessitating close multidisciplinary team follow-up with specific questions about HS. This service evaluation identified areas requiring improvement in diagnosis and monitoring, including temperature dysregulation and behaviour. Implementation of a standardised hypothalamic syndrome checklist could improve recognition and monitoring of these challenging late effects.