ISSN 1662-4009 (online)

ESPE Yearbook of Paediatric Endocrinology (2024) 21 1.13 | DOI: 10.1530/ey.21.1.13

Eur J Endocrinol. 2024 May 2;190(5):363-373. doi: 10.1093/ejendo/lvae044. PubMed: 38662730


Brief Summary: This single-centre retrospective cohort study offers valuable insights into the trajectory of weight gain in a paediatric craniopharyngioma cohort over a mean follow-up period of 10.4 years, reinforcing the necessity for targeted interventions to address this issue.

Craniopharyngioma poses a substantial clinical challenge in paediatric patients, primarily due to the risk of hypothalamic involvement. A particularly severe long-term consequence of treatment is hypothalamic obesity, characterized by excessive weight gain resulting from reduced basal metabolic rate and energy expenditure rather than increased energy intake. As a result, recent approaches have increasingly focused on hypothalamus-sparing treatments to prevent hypothalamic syndrome.

This study analysed data from 108 paediatric craniopharyngioma cases. It highlights that despite advances in hypothalamus-sparing surgical techniques, weight gain remains a major concern. Hypothalamic integrity plays a crucial role in controlling weight gain; however, even in cases where the hypothalamus was not involved, significant weight gain was still observed. This underscores the intrinsic challenge of managing hypothalamic obesity, which is less responsive to conventional lifestyle interventions due to the complex neuroendocrine dysregulation following hypothalamic damage.

Female sex, higher baseline BMI, and hypothalamic involvement by the tumour were key risk factors for greater BMI-SDS changes over time. This suggests that early intervention strategies should be personalised, particularly for high-risk groups, such as females and those presenting with a higher BMI at diagnosis. The observation that hypothalamic-sparing surgery, while beneficial, does not completely prevent weight gain, calls for ongoing exploration of adjunct therapies that target the metabolic disturbances related to hypothalamic obesity. The study also points to the need for novel therapeutic approaches.

This study underscores the need for long-term monitoring and a multidisciplinary strategy in managing paediatric craniopharyngioma, as hypothalamic obesity continues to be a significant challenge despite advances in surgical techniques

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