ESPE Abstracts

Introduction: Craniopharyngioma (CP) is a histologically benign rare tumor from the sellar and parasellar region. Its invasion into adjacent structures, namely optic nerve and hypothalamic-pituitary axis brings significant morbidity and warrants surgical treatment. Hypothalamic Syndrome (HS) can occur in different conditions affecting this structure, comprising a cluster of symptoms like pituitary dysfunction, obesity, temperature dysregulation, sleep disturbances, and behavioral problems. According to these domains, diagnostic criteria for HS in childhood were recently proposed.

Aim: To access HS in childhood-onset CP, before and after surgical treatment.

Methods: Retrospective observational study of CP patients followed in a tertiary pediatric hospital from January 2008 until December 2022. Collected variables: sex, age at diagnose, clinical presentation, therapeutical approach, clinical outcome.

Results: 10 individuals with CP were reviewed: 80% males, median age at onset of 6,5y (range: 0,8 - 15y). The most frequent onset signs were headache and vomiting (59%), visual impairment (50%), and pituitary hormonal deficits (50%). Of these, TSH and ACTH were diminished in 50% and 30% of the cases, respectively. Seizures (20%), hypersomnia (20%), cold intolerance (20%), failure to thrive (20%), and hyperphagia and obesity (10%) were also documented. 50% were approached endoscopically. All CP were of the adamantinomatous subtype. HS was present in 30% of the cases, all of them MRI Muller grading 2, recurring and needing posterior radiotherapy intervention. Until now, there is 100% survival. After surgery, HS was diagnosed in 60% of the cases: all of whom either needing open transcranial approach or adjunctive radiotherapy, or having a relapse. Hormonal deficits were present in all patients (TSH: 100%; ACTH: 80%; LH/FSH: 70%; ADH: 70%; GH: 60%). Central precocious puberty was not observed. Hyperphagia and obesity were present in 60% of the cases, all of them with documented insulin resistance and reports of temper outbursts related to food restriction. One child had sleep disturbances. There were no reports of cognitive impairment either before or after surgery.

Discussion: More severe cases increased the prevalence of HS after treatment. Whilst neuroendocrine consequences of craniopharyngiomas are well established, neuropsychological ones are less frequently reported and can be as important in these patients management. We hypothesize that some of these symptoms may be underrepresented and therefore its incidence might be undervalued. Further prospective studies and a multidisciplinary protocolled approach to these patients might improve these outcomes.