ESPE Abstracts (2024) 98 RFC15.3

ESPE2024 Rapid Free Communications Late Breaking (6 abstracts)

The different faces of acquired hypothalamic dysfunction.

Hanneke M. van Santen 1,2 , Ichelle van Roessel 1,2 & Sanne Hulsmann 1


1Princess Maxima Center, Utrecht, Netherlands. 2Wilhelmina Children's Hospital, Utrecht, Netherlands


Background: Acquired hypothalamic dysfunction (HD) in children following suprasellar brain tumor treatment may be caused by the tumor or its treatment (neurosurgery, radiotherapy). Rapid weight gain, obesity and pituitary dysfunction are readily recognized as symptoms of HD, however HD knows other signs and symptoms such as hypothermia, adipsia and behavioral problems. In our clinical experience, signs and symptoms of HD may differ per tumor type and the treatment given, but this has never been systematically assessed. We aimed to systematically describe three different suprasellar tumor cohorts known in our nationwide pediatric oncology center, to illustrate the multi-faceted character of HD.

Materials/methods: A retrospective analysis was done, including patients with a childhood suprasellar low-grade glioma (LGG), childhood craniopharyngioma (cCP), or a suprasellar germ cell tumor (GCT) diagnosed between 2003-2023 and known at our national pediatric oncology center. The diagnostic criteria for the hypothalamic syndrome (HS) as published by van Santen et al. were scored and compared.

Results: In total, 336 patients were included, of whom 220 with LGG, 88 with cCP, and 28 with GCT. Median follow-up time was 7.6, 7.5, and 3.5 years, respectively. In 15.0% (LGG), 12.5% (cCP) and 3.6% (GCT) of patients, (hypothalamic) overweight or obesity (HO) was present at tumor diagnosis without pituitary dysfunction. At last moment of follow-up, HS was found in 106 patients (35.9%, 48.8% and in 16.7% of the cohorts, respectively), but HS did not have the same “face” in all patients. In total, 45 different faces were observed, varying from dysfunction in all 6 hypothalamic domains to dysfunction in several domains, such as the combinations of panhypopituitarism, hyperphagia and obesity to behavioral- and sleep problems with hypothalamic obesity or temperature dysregulation, obesity and panhypopituitarism. HO was present in 21.8%, 41.4%, and 23.1%, respectively. Temperature dysregulation was more common in LGG (43.3%) and GCT (58.3%) cohorts, compared to the cCP cohort (19.7%).

Conclusion: Acquired HD has many different faces and concerns more than HO. Overweight can be caused by HD, even if the pituitary function is still intact. Temperature dysregulation is more common in LGG and GCT patients. In cCP cohort, HO is more often accompanied by hyperphagia and pituitary deficiencies. Recognition of the different faces of acquired HD at diagnosis and follow-up of a suprasellar tumor is important as it may help to prevent delay in diagnosis and open doors towards personalized interventions.

Volume 98

62nd Annual ESPE (ESPE 2024)

Liverpool, UK
16 Nov 2024 - 18 Nov 2024

European Society for Paediatric Endocrinology 

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